Articles de revues : « Mielodisplasie »

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Buccisano, Francesco, Anna Lina Piccioni, Carolina Nobile, Marianna Criscuolo, Pasquale Niscola, Caterina Tatarelli, Luana Fianchi et al. « Real-life use of erythropoiesis-stimulating agents in myelodysplastic syndromes : a “Gruppo Romano Mielodisplasie (GROM)” multicenter study ». Annals of Hematology 95, n^o 7 (19 avril 2016) : 1059–65. http://dx.doi.org/10.1007/s00277-016-2667-1.

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Neri, B., C. Nobile, A. L. Piccioni, R. Ricci, S. Mancini, M. Criscuolo, M. D'Andrea et al. « 381 A new point of view on myelodysplastic syndromes from a novel cooperative group in Italy (From : Gruppo Romano delle Mielodisplasie) ». Leukemia Research 35 (mai 2011) : S152. http://dx.doi.org/10.1016/s0145-2126(11)70383-6.

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Maurillo, Luca, Massimo Breccia, Francesco Buccisano, Maria Teresa Voso, Pasquale Niscola, Giulio Trapè, Caterina Tatarelli et al. « Deferasirox chelation therapy in patients with transfusion-dependent MDS : a ‘real-world’ report from two regional Italian registries : Gruppo Romano Mielodisplasie and Registro Basilicata ». European Journal of Haematology 95, n^o 1 (12 mars 2015) : 52–56. http://dx.doi.org/10.1111/ejh.12476.

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Mayer, Flavia, Laura Faglioni, Nera Agabiti, Susanna Fenu, Francesco Buccisano, Roberto Latagliata, Roberto Ricci et al. « A POPULATION-BASED STUDY ON MYELODYSPLASTIC SYNDROMES IN THE LAZIO REGION (ITALY), MEDICAL MISCODING AND 11-YEAR MORTALITY FOLLOW-UP : THE GRUPPO ROMANO-LAZIALE MIELODISPLASIE EXPERIENCE OF RETROSPECTIVE MULTICENTRIC REGISTRY ». Mediterranean Journal of Hematology and Infectious Diseases 9, n^o 1 (1 juillet 2017) : e2017046. http://dx.doi.org/10.4084/mjhid.2017.046.

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Results on myelodysplastic syndromes (MDS) from population-based studies are rare and these data are infrequently collected by cancer registries because diagnostic difficulties and under-reported data.Our is the first regional Lazio study about medical coding, diagnosis, classification and mortality of MDS patients. This study is aimed at evaluating MDS medical miscoding and conducting a mortality follow-up in a cohort of 644 MDS patients enrolled in the Gruppo Romano-Laziale Mielodisplasie (GROM-L) registry from 2002 to 2010.We linked the MDS cohort with 2 regional health information systems: the Hospital Information System (HIS) and the regional Mortality Information System (MIS).About the first objective 92% of the patients had at most 12 hospitalization, but 28.5% of them had no hospitalization with the 238.7 ICD-9-CM. About the second objective we observed 45.5% of death during the follow-up, Myelodysplastic Syndrome was the second cause of death, other frequent causes of death were myeloid leukemia and aplastic anemia.This study highlights for the first time in Lazio that a disease like MDS, involving many resources for care assistance, tends to be under-documented in the HIS archive. This may be due to the evolution of the disease over the time, the inappropriate use of existing ICD-9-CM and the limitations of current ICD-9-CM classification. Moreover, the most frequent causes of death other than MDS might suggest a miscoding of MDS in the death causes too.In conclusion our registry could be a useful investigational tool to make a continued surveillance on medical miscoding and collect epidemiological data.

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Voso, Maria Teresa, Susanna Fenu, Roberto Latagliata, Francesco Buccisano, Alfonso Piciocchi, Maria Antonietta Aloe-Spiriti, Massimo Breccia et al. « Revised International Prognostic Scoring System (IPSS) Predicts Survival and Leukemic Evolution of Myelodysplastic Syndromes Significantly Better Than IPSS and WHO Prognostic Scoring System : Validation by the Gruppo Romano Mielodisplasie Italian Regional Database ». Journal of Clinical Oncology 31, n^o 21 (20 juillet 2013) : 2671–77. http://dx.doi.org/10.1200/jco.2012.48.0764.

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Purpose The definition of disease-specific prognostic scores plays a fundamental role in the treatment decision-making process in myelodysplastic syndrome (MDS), a group of myeloid disorders characterized by a heterogeneous clinical behavior. Patients and Methods We applied the recently published Revised International Prognostic Scoring System (IPSS-R) to 380 patients with MDS, registered in an Italian regional database, recruiting patients from the city of Rome (Gruppo Romano Mielodisplasie). Patients were selected based on the availability of IPSS-R prognostic factors, including complete peripheral-blood and bone marrow counts, informative cytogenetics, and follow-up data. Results We validated the IPSS-R score as a significant predictor of overall survival (OS) and leukemia-free survival (LFS) in MDS (P < .001 for both). When comparing the prognostic value of the International Prognostic Scoring System (IPSS), WHO Prognostic Scoring System (WPSS), and IPSS-R, using the Cox regression model and the likelihood ratio test, a significantly higher predictive power for LFS and OS became evident for the IPSS-R, compared with the IPSS and WPSS (P < .001 for both). The multivariate analysis, including IPSS, WPSS, age, lactate dehydrogenase, ferritin concentration, Eastern Cooperative Oncology Group performance status, transfusion dependency, and type of therapy, confirmed the significant prognostic value of IPSS-R subgroups for LFS and OS. Treatment with lenalidomide and erythropoiesis-stimulating agents was shown to be an independent predictor of survival in the multivariate analysis. Conclusion Our data confirm that the IPSS-R is an excellent prognostic tool in MDS in the era of disease-modifying treatments. The early recognition of patients at high risk of progression to aggressive disease may optimize treatment timing in MDS.

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Tudury, Eduardo Alberto, Pedro Luís de Camargo, Dominguita Luhers Graça et Otavio Pedro Neto. « Mielodisplasia segmentar múltipla em gato ». Ciência Rural 30, n^o 3 (juin 2000) : 529–31. http://dx.doi.org/10.1590/s0103-84782000000300028.

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Relata-se o caso de uma gata Siamês de seis meses de idade que desde o nascimento apresentava paraplegia espástica associada à incontinência fecal e urinária. No exame neurológico, exibia paralisia, analgesia, hiperreflexia e perda das reações posturais nos membros posteriores. Radiografias simples e contrastadas (mielografia) da coluna vertebral toracolombar evidenciaram estreitamentos do canal vertebral na região lombar e da medula espinhal, vértebras torácicas e lombares. Na necropsia e exame histopatológico, verificou-se hipoplasia segmentar caracterizada pelo afinamento localizado da medula espinhal em T4-T6 e L2-L3, depleção neuronal na substância cinzenta e filetes nervosos interligando as partes craniais e caudais, todos recobertos por dura mater íntegra e espessada. Essas alterações permitiram concluir o diagnóstico de mielodisplasia segmentar múltipla.

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Nindhita, Like Rahayu, et Dian Widyaningrum. « Immunoassay interference : Studi kasus Nefritis Lupus kelas IV, Sindroma Mielodisplasia dan Neuropsikiatrilupus ». Medica Hospitalia : Journal of Clinical Medicine 8, n^o 2 (15 juillet 2021) : 252–64. http://dx.doi.org/10.36408/mhjcm.v8i2.610.

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Latar belakang : Systemic Lupus Erythematosus adalah penyakit kronik inflamatif autoimun dengan manifestasi multiorgan. Pemeriksaan bone marrow punction mengkonfirmasi gangguan hematologi. Imunoassai ANA, anti-dsDNA dan anti Sm mengkonfirmasi imunopatogenesis. Tujuan studi kasus ini adalah untuk menganalisis aspek laboratorium dalam diagnosis dan tatalaksana nefritis Lupus kelas IV dan neuropsikiatri lupus dengan Sindroma Mielodisplasia. Kasus : Seorang wanita 35 tahun mengeluh lemas. Pemeriksaan fisik : hipertensi, konjungtiva palbebra pucat, ulkus dimulut, echimosis dikulit, pleural friction rub paru kanan, edema pitting ekstremitas. Pemeriksaan laboratorium : peningkatan LDH, hiperlipidemia, hipofibrinogenemia, peningkatan D-dimer, proteinuria, hematuria dan silinderuria. Pemeriksaan ANA dan Sel LE positif. Pansitopenia darah tepi dikonfirmasi pemeriksaan BMP didapatkan gambaran MDS. Pasien mengalami kejang, dikonfirmasi MSCT kepala terdapat infark serebri multipel. Anti ds-DNA positif moderate, C3/C4 rendah namun anti Sm negatif. Kesimpulan : Berdasarkan data klinis dan laboratoris pasien ini didiagnosis SLE derajat berat, nefritis lupus kelas IV, sindroma mielodisplasia dan neuropsikiatrilupus. Anti Sm negatif kemungkinan disebabkan fenomena “cross reactivity” atau “Hook Effect” sehingga menyebabkan interferensi hasil negatif palsu. Anti Sm negatif tidak dapat menyingkirkan diagnosis SLE

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Osipov, I. B., S. A. Sarychev, D. A. Lebedev et A. Yu Shchedrina. « Strukturirovanie urologicheskikh proyavleniy kaudal'noy mielodisplazii u detey ». Urologicheskie vedomosti 5, n^o 1 (15 mars 2015) : 83. http://dx.doi.org/10.17816/uroved5183-84.

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Luz, Flávia S., Renata D. Mazaro, Douglas M. Lorensetti, Glaucia D. Kommers, Mariana M. Flores et Rafael A. Fighera. « Mielodisplasia na peritonite infecciosa felina : 16 casos (2000-2017) ». Pesquisa Veterinária Brasileira 38, n^o 8 (août 2018) : 1638–48. http://dx.doi.org/10.1590/1678-5150-pvb-5578.

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RESUMO: Apesar da prevalência da peritonite infecciosa felina (PIF) ser alta em praticamente o mundo todo, estudos anatomopatológicos recentes acerca dessa doença são escassos. Não obstante, as características microscópicas da medula óssea de gatos com PIF estão ausentes da literatura consultada. O objetivo deste artigo é descrever alterações medulares ósseas vistas em casos espontâneos de PIF. As medulas ósseas colhidas sistematicamente da região diafisária dos fêmures de 16 gatos necropsiados no Laboratório de Patologia Veterinária (LPV) da Universidade Federal de Santa Maria (UFSM), Rio Grande do Sul, entre janeiro de 2000 e junho de 2017, e que tiveram diagnóstico definitivo de PIF, foram avaliadas fenotípica (histopatologia - hematoxilina e eosina e histoquímica - reação de Perls) e imunofenotipicamente (utilizando marcadores mieloides (anti-MAC387) e de linfócitos (anti-CD79αcy e anti-CD3). Os resultados permitem afirmar que, independentemente da apresentação clinicopatológica da doença ocorrem as seguintes alterações: 1) hiperplasia mieloide; 2) hipoplasia eritroide, 3) displasia megacariocítica (dismegacariocitopoiese) e 4) plasmocitose medular. Exclusivamente nos casos de PIF seca há hemossiderose medular óssea e hepática. Essas alterações permitem estabelecer que gatos com PIF desenvolvem mielodisplasia, uma lesão mieloproliferativa muito semelhante àquela relatada em humanos infectados pelo HIV. Sugere-se que a partir dos achados aqui descritos, a mielodisplasia seja considerada a principal responsável pelas alterações hematológicas observadas na PIF, especialmente pela anemia e trombocitopenia arregenerativas frequentemente desenvolvidas pelos pacientes com essa doença.

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Latagliata, Roberto, Maria Antonietta Aloe Spiriti, Luca Maurillo, Carolina Nobile, Anna Lina Piccioni, Marianna Criscuolo, Virginia Naso et al. « Response to Erythropoietin in a Multicentric Real-Life Cohort of Myelodysplastic Patients : The Grom Experience ». Blood 120, n^o 21 (16 novembre 2012) : 4958. http://dx.doi.org/10.1182/blood.v120.21.4958.4958.

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Abstract Abstract 4958 Erythropoietin (EPO) have been widely employed in the treatment of patients with low-risk Myelodysplastic Syndromes (MDS) and anemia, with response rates ranging from 30 to 60%. These data, however, have been derived only from controlled clinical trials or unicentric single-arm studies; it is still lacking a wider survey evaluating the use of EPO in the real-life clinical practice. To address this issue, the Gruppo Romano Mielodisplasie (GROM) revised retrospectively 394 MDS patients (M/F 225/169, median age at diagnosis 73. 9 yrs, IR 67. 0 – 79. 3) treated with EPO from 1/2002 to 12/2010 by 11 Hematological Centers (5 university hospitals and 6 community-based hospitals) in the metropolitan area of Rome. According to WHO classification, there were 81 (20. 6%) patients with RA, 7 (1. 8 %) with SA, 160 (40. 7%) with RCMD, 17 (4. 3%) with RCMD-S, 75(19. 0%) with RAEB-1, 27 (6. 8%) with RAEB-2 and 27 (6. 8%) with isolated del5q. The IPSS score was calculated in the 307 patients with an available karyotype: 145 (47. 2%) patients were low-risk, 135 (44. 0%) int-1, 24 (7. 8%) int-2 and 3 (1. 0%) high-risk. Median interval from diagnosis to EPO start was 3. 7 months (IR 0. 9 – 12. 1). At EPO start, median age was 74. 5 yrs (IR 68. 3 – 79. 9) with a median haemoglobin level of 8. 9 g/dl (IR 8. 2 – 9. 6). Creatinine level was elevated in 64 (16. 2%) cases: 138 patients (35. 3%) had a previous transfusion requirement. Median serum EPO level was 50. 0 mU/L (IR 26. 2 – 110. 0). The initial doses of EPO were ≤ 40. 000 UI/week in 259 patients (65. 7%) (standard doses, α-EPO in 104 patients, β-EPO in 143 patients, darbepoietin in 12 patients) and 80000 UI/week in 135 patients (34. 3%) (high doses, α-EPO in 130 patients, β-EPO in 5 patients). An erythroid response was observed in 228 (57. 9%) patients, with Hb increase > 1. 5 g/dl in 210 patients (53. 3%) and disappearance of transfusion requirement in 18 (4. 6%): patients receiving initial high doses had a higher response rate compared to patients receiving standard doses [94/135 (69. 6%) vs 134/259 (51. 7%), p=0. 002]. Only 5 thrombotic events (1. 2%) were reported during the treatment. Predicting factors for erythroid response were no previous transfusion requirement (p<0. 001), serum EPO levels at baseline < 50 mU/l (p<0. 001), creatinine levels above the normal values (<0. 001), ferritin levels < 250 ng/ml (p=0. 009) and hemoglobin level at baseline > 8 g/dl (p=0. 017). Median overall survival from EPO start was 70. 7 months (CI 95% 52. 5 – 88. 8) in responders versus 41. 7 months (CI 95% 27, 6 – 55, 7) in resistant patients (p= 0. 018). Our real-life data from a single homogeneous geographic area outline that EPO treatment is safe and effective also in the current clinical practice, beyond controlled clinical trials. However, this latter type of studies is warranted to define the best initial dose of EPO in such patients. Disclosures: No relevant conflicts of interest to declare.

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Silva, C. S., D. S. Sant’ana, M. P. Araujo, P. A. D. S. B. A. Matos, M. D. D. Santos, S. S. Marcondes, M. S. Teixeira, G. S. Sonsim, V. H. R. Carvalho et W. L. D. Reis. « RELATO DE CASO : ASPERGILOSE INVASIVA E COVID-19 EM PACIENTE COM MIELODISPLASIA ». Hematology, Transfusion and Cell Therapy 42 (novembre 2020) : 554. http://dx.doi.org/10.1016/j.htct.2020.10.935.

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Oktariani, Siswi, Adeodatus Yuda Handaya et Ibnu Purwanto. « Peningkatan profil klinik-hematologi pada pasien sindrom mielodisplasia multilini paska splenektomi total ». Jurnal Penyakit Dalam Udayana 4, n^o 1 (20 juin 2020) : 1–4. http://dx.doi.org/10.36216/jpd.v4i1.133.

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Myelodysplasia syndrome (MDS) is a hematologic disorder manifested by cytopenia and rarely with splenomegaly. Clinical complaints that often accompany symptoms of anemia and when experiencing splenomegaly, the patient feels a full stomach or sometimes pain in the upper left abdomen. We report a case of a 55 year old woman with a full stomach complaint with a history of thrombocytopenia. From the peripheral blood examination, observation of thrombocytopenia was obtained with suspicion of the inflammatory process and physical examination of splenomegaly. Bone marrow aspiration studies revealed a multi-line cytopenic refractory MDS. Patients receive immunosuppressant therapy without blood transfusions. After 1 year of treatment the patient complained of fatigue and upper left abdominal pain. The results showed pancytopenia and splenomegaly. The doctor and patient discussion agreed to the splenectomy. The patient had no complications after splenectomy and for 11 months the patient felt complaint free and normal hematological parameters with a good quality of life. This case report suggests that splenectomy can be an alternative therapy for MDS that is refractory to previous therapy and is complaining of splenomegaly. Splenectomy can improve the clinical complaints and hematologic profile of MDS patients with splenomegaly.

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Santos, Fabiana Azevedo. « Avaliação da Abordagem Fisioterapêutica no Tratamento de Paciente Pediátrico Portador de Mielomeningocele ». Revista de Saúde 1, n^o 1 (1 mars 2010) : 01. http://dx.doi.org/10.21727/217927392010.rs.v1i1.01-06.

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Mielomeningocele é uma anomalia congênita complexa da coluna que causa vários graus de malformação da coluna espinal, ou mielodisplasia. É comumente referida como espinha bífida e é classificada como um defeito do tubo neural. Pacientes com essa desordem apresentam-se com um espectro de imperfeições, mas os déficit funcionais primários são a paralisia da coluna lombar e a perda sensorial, disfunção da bexiga e do intestino, e a disfunção cognitiva. Problemas médico-cirúrgicos e de reabilitação surgem no paciente com mielomeningocele desde o nascimento até a idade adulta. Esse trabalho apresentará a avaliação da terapia física adaptada no tratamento de pacientes pediátricos que possuam mielomelingocele.

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Santos, Fabiana Azevedo. « Avaliação da Abordagem Fisioterapêutica no Tratamento de Paciente Pediátrico Portador de Mielomeningocele ». Revista de Saúde 1, n^o 1 (1 mars 2010) : 01. http://dx.doi.org/10.21727/rs.v1i1.113.

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Mielomeningocele é uma anomalia congênita complexa da coluna que causa vários graus de malformação da coluna espinal, ou mielodisplasia. É comumente referida como espinha bífida e é classificada como um defeito do tubo neural. Pacientes com essa desordem apresentam-se com um espectro de imperfeições, mas os déficit funcionais primários são a paralisia da coluna lombar e a perda sensorial, disfunção da bexiga e do intestino, e a disfunção cognitiva. Problemas médico-cirúrgicos e de reabilitação surgem no paciente com mielomeningocele desde o nascimento até a idade adulta. Esse trabalho apresentará a avaliação da terapia física adaptada no tratamento de pacientes pediátricos que possuam mielomelingocele.

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Santos, Fabiana Azevedo. « Avaliação da Abordagem Fisioterapêutica no Tratamento de Paciente Pediátrico Portador de Mielomeningocele ». Revista de Saúde 1, n^o 1 (27 septembre 2016) : 01. http://dx.doi.org/10.21727/rs.v1i1.29.

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Mielomeningocele é uma anomalia congênita complexa da coluna que causa vários graus de malformação da coluna espinal, ou mielodisplasia. É comumente referida como espinha bífida e é classificada como um defeito do tubo neural. Pacientes com essa desordem apresentam-se com um espectro de imperfeições, mas os déficit funcionais primários são a paralisia da coluna lombar e a perda sensorial, disfunção da bexiga e do intestino, e a disfunção cognitiva. Problemas médico-cirúrgicos e de reabilitação surgem no paciente com mielomeningocele desde o nascimento até a idade adulta. Esse trabalho apresentará a avaliação da terapia física adaptada no tratamento de pacientes pediátricos que possuam mielomelingocele.

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Quito Ochoa, Paola Yessenia, Luz María Bojorque Bojorque, Andrea Melissa Márquez Torres, Germania Elizabeth Ortiz Freire et Santiago Martín Sánchez Peralta. « Manifestaciones hematológicas crónicas por exposición a benceno en despachadores de combustible ». Salud, Ciencia y Tecnología 2, S1 (30 décembre 2022) : 204. http://dx.doi.org/10.56294/saludcyt2022204.

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Introducción: El benceno es un hidrocarburo aromático considerado como cancerígeno por su alta toxicidad, aunque en los últimos años su exposición ha disminuido, sin embargo, no ha sido suficiente para prevenir complicaciones. La exposición crónica en concentraciones superiores o igual a 1 ppm por un periodo superior a 8 horas diarias durante 10 años constante o cuando su exposición supera los 10 ppm durante 1 año existe riesgo de desarrollar trastornos hematológicos. Objetivo: Describir las manifestaciones hematológicas crónicas por exposición a Benceno en despachadores de combustible.Método: Se realizó una revisión narrativa, recopilando información desde enero 2000, hasta noviembre de 2022 en base de datos científicas como Pubmed, Enviromental Pollution, Dialnet, SAGE, Taylor and Francis, Springerlink, Science Direct, Scielo; empleando palabras claves. Resultados: Las manifestaciones hematológicas que se desarrollan tras la exposición crónica de benceno en cualquier concentración se encuentran la leucemia linfoide, linfoma no Hodgkin, leucemia mieloide aguda, síndrome mielodisplasico, leucemia mieloide crónica, mieloma múltiple, leucemia mieloide crónica y anemia aplásica. Conclusiones: Existen diversas manifestaciones tras la exposición crónica de benceno destacando leucemia linfática crónica, linfoma no Hodgkin, leucemia mieloide aguda o crónica, síndrome mielodisplasico y mieloma múltiple; es por ello que se recomienda hacer controles médicos anuales y la aplicación de estrategias que reduzcan el tiempo de exposición laboral, de forma que permitan diagnosticar y prevenir enfermedades hematológicas ya que actualmente no existen normativas de prevención que garanticen el bienestar de los despachadores.

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Cruz, LN, CE Avila, DRN Garcia, TA Bonilha, RSP Silva, AMB Azevedo, AM Sousa, MGP Land, APS Bueno et DT Vianna. « LEUCEMIA MIELOIDE AGUDA RELACIONADA À MIELODISPLASIA COM TROMBOCITOSE EM PACIENTE PEDIÁTRICO : RELATO DE CASO ». Hematology, Transfusion and Cell Therapy 44 (octobre 2022) : S353. http://dx.doi.org/10.1016/j.htct.2022.09.596.

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Zanchetta, Lais Aparecida Branco, Letícia Rufino Artuso, Mariana Longo Moraes, Mariana Santos Teixeira, Melina Tavares Di Trani, Saulo Duarte Passos et Marcia Borges Machado. « RELATO DE CASO ‐ SÍNDROME INFLAMATÓRIA MULTISSISTÊMICA PEDIÁTRICA EM PACIENTE PORTADORA DE MIELODISPLASIA COM CITOPENIAS REFRATÁRIAS ». Brazilian Journal of Infectious Diseases 25 (janvier 2021) : 101164. http://dx.doi.org/10.1016/j.bjid.2020.101164.

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Savioli, M., F. Spagnoli, M. A. Aloe Spiriti, M. C. Petti, R. Latagliata, D. Bertelletti, R. Villa et F. Mandelli. « Tests of rHuEPO administration in patients affected by mielodisplastic syndrome endocrino-metabolic evaluation ». Leukemia Research 15 (janvier 1991) : 33. http://dx.doi.org/10.1016/0145-2126(91)90454-2.

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Grille, S., A. Brugnini, E. Velloso DRP, V. Cismondi, E. Jensen, N. S. Bacal, L. Solari et al. « Flow Cytometry “Ogata Score” in Latin America. On Behalf of the Grupo Latinoamericano De Mielodisplasia (GLAM) ». Leukemia Research 55 (avril 2017) : S97. http://dx.doi.org/10.1016/s0145-2126(17)30269-2.

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Balleari, Enrico, Chiara Salvetti, Andrea Bacigalupo, Gianluca Forni, Marco Gobbi, Riccardo Ghio, Marco Scudeletti et al. « The Impact of Comorbidities on Clinical Outcome of Patients with Myelodysplastic Syndromes : A Real-Life Survey ». Blood 124, n^o 21 (6 décembre 2014) : 4668. http://dx.doi.org/10.1182/blood.v124.21.4668.4668.

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Abstract Introduction: Myelodysplastic syndromes (MDS) are a highly heterogeneous group of clonal disorders, with very different prognosis in given individuals, overall survival (OS) ranging from more than 10 years (y) for the more indolent conditions to only few months (m) for the forms approaching AML; beside of the well-established disease-related prognostic systems (classical IPSS or its revised form [IPSS-R], the prognostic implication of comorbidities is emerging as a relevant patient-related factor influencing clinical outcome. Aim of our study was to evaluate the clinical impact of comorbidities in a series of MDS patients whatever treated in a “real-life” setting. Methods: this retrospective cohort study involved the MDS patients consecutively registered between Jan 2011 and Dec 2013 into the Registro Ligure delle Mielodisplasie database, a regional registry established within the framework of the Italian Network of regional MDS registries. Data of 318 patients (pts) with available complete assessment of comorbidities at diagnosis were included into the study. The clinical characteristics and comorbidities were all considered into the analysis. Comorbidities were evaluated according to both hematopoietic cell transplantation-specific comorbidity index (HCT-CI) and MDS-specific comorbidity index (MDS-CI). All survival analyses were made from the date of diagnosis to last follow-up, death, or progression to AML. Unless specified, survival analyses were Cox models using continuous variables accounting for interactions. Results: Our cohort mainly consisted of older (median age 75y (range 40-98) “lower-risk” MDS pts: according to IPSS stratification, 151 (54.7%) pts were classified as low-risk, 86 (31.2%) as intermediate-1, 32 (11.6%) as intermediate-2 and 7 (2.5%) were in the high-risk group. One or more comorbidity of any grade of severity was seen in 177 (55.7%) pts at diagnosis. The more common comorbidity was cardiac (26.5%). At least a single comorbidity was present in 61.2% of pts older than 75y and in 50.6% of younger pts (p=0.07). Cardiovascular disorders were more frequent among older (32.9% for >75y vs 15.1% for ≤ 75y, p<0.001), and among males (28.7% vs 17.1% for females, p=0.02). According to HCT-CI risk stratification, 141pts (44.3%) were in the low-risk group, 94 (29.6%) in the intermediate-risk group, and 83 (26.1%) in the high-risk group, while according to MDS-CI, 197 (61.9%) pts had a low-risk score, 99 (31.1%) were intermediate, and 22 (6.9%) were in the high-risk group. MDS-CI score was higher among males (43.8% vs 30.7% for females, p=0.02). It was also higher among subjects >75 y (48% vs. 28.9% for < 75 y (p=0.001). A lower comorbidity score impacted on the clinical choice for active forms of therapy, while pts with an higher burden of comorbidities were preferentially treated with supportive care, even if difference did not reach significance (p=0.07). Overall survival and risk of non-leukemic death (NLD) were analyzed (median f.u. 26.9 m (range 1-220). HCT-CI did not significantly correlated with OS nor NLD (p= 0.1 and p= 0.07, respectively), while MDS-CI was found to be of prognostic significance both for OS (mean 136.6 (95%CI 116-157) m for the low-risk group, 81.3 (95%CI 61-102) m for the intermediate group and 48.1 (95%CI 30-66) m for the high-risk group, p=0.001) and for NLD (mean 159.6 (95%CI 139-180) m for the low-risk group, 96.5 (95%CI 72-121) m for the intermediate group and 49 (95%CI 31-67) m for the high-risk group (p<0.001). The correlation was significant (p<0.001) in IPSS or IPSS-R “lower-risk” (low and intermediate-1 risk or very-low, low and intermediate groups, respectively) but not in IPSS nor IPSS-R “higher-risk” (intermediate-2 and high or high and very-high groups, respectively) pts. In multivariate analysis, the prognostic impact of MDS-CI remained independent of baseline IPSS (p=0.01) or IPSS-R (p=0.03). Conclusions: a comprehensive evaluation of comorbidities according to a tailored tool such is MDS-CI helps to predict survival in patients with MDS and should be incorporate to current prognostic scores in order to better define clinical management of these patients. Disclosures No relevant conflicts of interest to declare.

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Стародуб, Г. С., О. В. Басова, Н. В. Горяінова, Н. М. Третяк et А. І. Гордієнко. « The relationship of clinical and haematological and immune parameters in patients with mielodisplastic syndrome ». Family Medicine, n^o 3(65) (16 octobre 2016) : 26–29. http://dx.doi.org/10.30841/2307-5112.3(65).2016.79965.

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SALOMÃO, J. FRANCISCO, ANTÔNIO R. BELLAS, RENÊ D. LEIBINGER, ANA PAULA A. BARBOSA et MARIA ANNA P. B. BRANDÃO. « Malformação de Chiari do tipo II sintomática ». Arquivos de Neuro-Psiquiatria 56, n^o 1 (mars 1998) : 98–106. http://dx.doi.org/10.1590/s0004-282x1998000100016.

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Os autores analisam uma série de 17 crianças portadoras de mielodisplasias que desenvolveram sinais e sintomas da malformação de Chiari do tipo II. De acordo com a idade, dois grupos ficaram bem definidos: Grupo I, crianças no primeiro ano de vida, em que predominaram sinais e sintomas de comprometimento do tronco encefálico e nervos cranianos bulbares (n=13); Grupo II, composto por crianças com idade superior a um ano, em que as principais manifestações foram dor cervical e sinais cerebelares (n=4). O resultado do tratamento cirúrgico nos dois grupos foi distinto: enquanto a mortalidade no Grupo I atingiu 46,1%, nenhum paciente do Grupo II veio a falecer. O tratamento inicial consistiu na instalação ou revisão de derivação ventricular, sendo a descompressão crânio-vertebral reservada àqueles que não se beneficiaram com esses procedimentos. Os autores enfatizam a necessidade do imediato reconhecimento e tratamento do quadro, de modo a se obter resultados satisfatórios.

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Lunesia, Rangga, et Irza Wahid. « CHRONIC MIELOMONOSITIC LEUKEMIA PADA USIA MUDA ». Jurnal Kesehatan Andalas 7 (29 juillet 2018) : 54. http://dx.doi.org/10.25077/jka.v7i0.828.

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Chronic Mielomonositic Leukemia (CMML) adalah keganasan heterogen yang ditandai dengan monositosis di darah perifer (>1x109/L), terdapat kelainan mielodisplastik dan mieloproliferatif di sumsum tulang, dan memiliki kecendrungan untuk bertransformasi menjadi leukemia myeloid akut. Angka kejadian CMML sangat jarang, di Indonesia tercatat hanya 2,5 % kasus dari keseluruhan MDS. Pengobatan pada MP-CMML dengan jumlah blast sedikit adalah pemberian terapi sitoreduktif yaitu hydroxyurea yang bertujuan mengontrol proliferasi myelomonositic sel dan mengurangi organomegali. Telah dilaporkan suatu kasus laki-laki 28 tahun dengan keluhan pucat. Pada pemeriksaan fisik ditemukan konjungtiva anemis dan splenomegali. Pemeriksaan laboratorium didapatkan hemoglobin 6.5 gr/dl, leukosit: 93.540/mm3, hitung jenis: 1/0/6/42/8/27. Pemeriksaan BMP menunjukkan aktivitas Granulopoeitik: meningkat, ditemukan mieloblast 3%, Promielosit 3%, monoblast 2%, Promonosit 1%, dominasi monosit 33%, ditemukan juga dysplasia seperti pseudo pelgerhuet, dengan kesan sesuai gambaran Chronic Mielomonositic Leukemia. Pada pasien ini dengan jumlah leukosit > 13 x 109/L termasuk kedalam MP-CMML diberikan terapi hydroxyurea. Follow up setelah pemberian terapi Hb 8.8 gr/dl, Leukosit 38.200/mm3, pada hitung jenis: 0/1/5/45/7/23.

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Lozano-Masdemont, B., O. Baniandrés-Rodríguez, V. Parra-Blanco et R. Suárez-Fernández. « Dermatitis granulomatosa como manifestación cutánea de trastornos hematológicos : primer caso asociado a policitemia vera y un nuevo caso asociado a mielodisplasia ». Actas Dermo-Sifiliográficas 107, n^o 5 (juin 2016) : e27-e32. http://dx.doi.org/10.1016/j.ad.2015.05.017.

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Ruiz, Oscar, Carla Moore, John Rojas, David Díaz, Carlos Peña, Manuela Marangoni et Carlos Delgado. « Inmunofenotipo de leucemias agudas del Hospital Nacional Dos de Mayo, durante el periodo 2011-julio 2012 ». Anales de la Facultad de Medicina 73 (7 mai 2013) : 40. http://dx.doi.org/10.15381/anales.v73i1.2198.

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Objetivos: Determinar la prevalencia de leucemias agudas, por inmunofenotipo. Diseño: Descriptivo transversal. Institución: Instituto de Investigaciones Clínicas, Facultad de Medicina, UNMSM, y Hospital Nacional Dos de Mayo (HNDM). Participantes: Pacientes con neoplasias hematológicas atendidos en el HNDM en el periodo 2011-julio 2012. Intervenciones: A todos los pacientes se realizó hemograma, mielograma, citometría de flujo (sangre medular). Principales medidas de resultados: Prevalencia de neoplasias hematológicas y leucemias agudas, características inmunofenotípicas. Resultados: Se encontró 80 neoplasias hematológicas: 42 (52,5%) leucemias agudas, 21(26,3%) mieloma múltiple, 8 (10%), linfomas, 5 (6,3%) síndromes mieloproliferativos crónicos, 4 (5%) mielodisplasias. Las leucemias agudas con citometría, en 12 (41,4%) mujeres y 17 (58,6%) varones se encontró lla 17 (40,5%), 12 (28,6%) lma, 13 (30,9%) no tipificada. Características inmunofenotipicas de las lla: 2 fueron t y 15 b. Orden de frecuencia: lla común pura (10), lla pre b (02), lla pro b (02) lla bifenotípica (01). En lma: (12) lma–m2 (08), lma-m0 (1), lma-m1 (1), lma–m3 (1), lma-m5 (1). Conclusiones: 52% de las neoplasias en el HNDM fueron leucemias agudas. La lla b común fue la más frecuente (58,8% respecto a lla y 34,5% a todas las leucemias agudas con citometría), prevalencia similar a otros estudios internacionales.

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Palacio Lavid, Manuela, Patricia Jaramillo Arbelaez et Kenny Galvez. « Hallazgos morfológicos en síndromes mielodisplásicos ». Revista Colombiana de Hematología y Oncología 8, n^o 1 (1 octobre 2021) : 62–75. http://dx.doi.org/10.51643/22562915.117.

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Los Síndromes Mielodisplásicos (SMD) son un grupo heterogéneo de neoplasias clonales de las células hematopoyéticas de la médula ósea, las cuales generan alteracionesuncionales, citopenias, hemopoyesis ineficaz y por ende anemia, infecciones recurrentes y sangrado. Esta revisión se realizó con el propósito de resaltar la importancia de la evaluación microscópica de los cambios morfológicos en las diferentes líneas mieloides. Desde 2016 la OMS los clasificó así: con displasia unilinaje, con sideroblastos en anillo, con displasia multilinaje, con exceso de Blastos tipo 1 y tipo 2, con deleción aislada del 5q, no clasificable y SMD de la infancia y citopenia refractaria de la infancia. El diagnóstico se realiza con diferentes metodologías, como el inmunofenotipo por citometría de flujo, la citogenética y las displasias morfológicas en sangre periférica y médula ósea, las cuales revelan el tipo de mielodisplasia, y el pronóstico del paciente. Las displasias se presentan en una o más líneas celulares y son más relevantes los cambios en el núcleo, como la cariorrexis, los puentes internucleares en la línea eritroide, la alteración en la lobulación, forma y número de apéndices cromatínicos en granulocitos. El citoplasma puede presentar depósitos de hierro como sideroblastos en anillo, hipogranulación, gránulos aberrantes tipo Chediak, granulaciones tóxicas o mixtas y la presencia de micromegacariocitos. Además, puede presentar tamaños pequeños y cambios megaloblásticos. Los cambios morfológicos en periferia son el primer acercamiento al diagnóstico de displasia, razón por la cual se requiere fortalecer la lectura de sangre periférica y médula ósea en 100X, asociada a los conteos celulares, con el fin de realizar un diagnóstico oportuno del Síndrome Mielodisplásico.

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Silveira, ACA, NCC Oliveira, FF Andrade, CAT Alves, K. Urago et RD Portugal. « MDS COM FIBROSE (MDS-F) EVOLUINDO COM LMA RELACIONADA À MIELODISPLASIA (MUTAÇÃO EM ZRSR-2) ANALISADO À LUZ DA NOVA CLASSIFICAÇÃO DA WHO 5ªEDIÇÃO - 2022 ». Hematology, Transfusion and Cell Therapy 44 (octobre 2022) : S206—S207. http://dx.doi.org/10.1016/j.htct.2022.09.348.

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Gálvez, Kenny Mauricio, María Helena Solano, Carlos Alberto Ramírez, Virginia Abello, Enrique Pedraza, Hermann Esguerra, Carmen Rosales, Licet Villamizar et Jesús Antonio Reyes. « Terapia primaria en aplasia de médula ósea : Trasplante alogénico de progenitores hematopoyéticos vs. terapia inmunosupresora ». Revista Repertorio de Medicina y Cirugía 16, n^o 3 (1 septembre 2007) : 143–50. http://dx.doi.org/10.31260/repertmedcir.v16.n3.2007.470.

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Antecedentes: la aplasia de médula ósea (AMO) es una enfermedad poco frecuente caracterizada por un síndrome de falla medular con citopenias periféricas y médula ósea hipo o acelular, en ausencia de blastos y mielodisplasia. El trasplante alogénico de progenitores hematopoyéticos (TAPH) y la terapia inmunosupresora (TI) son tratamientos aceptados para pacientes con esta patología. Objetivo: determinar la supervivencia global a un año de los pacientes con AMO de acuerdo con el tratamiento recibido. Diseño: estudio de serie de casos. Lugar: servicio de hematología del Hospital de San José y unidad de trasplante de médula ósea de la Clínica de Marly. Pacientes: 36 con AMO. Intervención: trasplante alogénico de progenitores hematopoyéticos de un donante HLA-idéntico relacionado o terapia inmunosupresora. Medición: se realizó el análisis descriptivo a través de tablas de frecuencia. Las probabilidades de supervivencia fueron estimadas usando el método de Kaplan-Meier. Resultados: la supervivencia global a un año del grupo de TAPH fue de 64% y del grupo de TI 57%. Respecto a los factores pronósticos, se encontró que en los dos grupos de tratamiento los recuentos de neutrófilos >500 y de plaquetas >10.000 al momento del diagnóstico se relacionaron con una mayor supervivencia en el primer año. Además un menor soporte transfusional tanto de PQ como de glóbulos rojos desde el diagnóstico hasta el momento del tratamiento tiene una fuerte relación con la supervivencia al año. Conclusión: en los pacientes con AMO el TAPH y la TI conllevan tasas de supervivencia a un año muy similares. Abreviaturas: AMO, aplasia de médula ósea; PQ, plaquetas; TAPH, trasplante alogénico de progenitores hematopoyécticos; TI, terapia inmunosupresora; HLA, antígeno mayor de histocompatibilidad.

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Indrasari, Yulia Nadar, et Ana Murtasyidah. « ACUTE MEGAKARYOBLASTIC LEUKEMIA ». INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY 25, n^o 3 (25 avril 2019) : 364. http://dx.doi.org/10.24293/ijcpml.v25i3.1503.

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Leukemia megakarioblastik akut (AMegL) dibagi dalam tiga kelompok berdasarkan patofiosiolgi, usia, respon terhadap terapi dan prognosis. Kelompok tersebut adalah AMegL yang terjadi pada anak-anak dengan sindrom Down (DS-AMegL), AMegL yang terjadi pada anak-anak yang tidak memiliki sindrom Down (non-DS-AMegL) dan AMegL pada orang dewasa non-DS (AMegL dewasa). AMegL pada anak tanpa sindrom Down juga disebut leukemia megakarioblastik pediatrik akut atau AMegL anak.1Dasar diagnosis AMegL atau AML M7 menurut FAB adalah adanya sel lini megakariosit 30% atau lebih dari seluruh sel.2 Sedangkan diagnosis AMegL menurut panduan WHO 2016 adalah leukemia akut dengan > 20% blast dimana > 50% adalah lini megakariosit. Sel megakariosit lebih jelas terlihat pada mikroskop elektron yang bereaksi positif terhadap platelet peroksidase2 atau menggunakan antibodi marker terhadap CD41/gpIIb, CD42b/gpIb, CD61/gpIIIa, faktor Von Willebrand dan pengecatan LAT.3 Temuan sitogenetika berbeda antara ketiga jenis AMegL sesuai dengan perbedaan patofisiologinya. WHO (2016) menyebutkan leukemia megakarioblastik akut ke dalam kriteria AML not otherwise specific (NOS). AmegL adalah leukemia akut dengan > 20% blast dimana > 50% adalah lini megakariosit. Kriteria ini mengeksklusi AML dengan mielodisplasia (acute myeloid leukemia with myelodysplasia related change; AMLMRC), AML yang berhubungan dengan terapi, dan AML dengan kelainan genetik rekuren, seperti AML dengan t(1;22)(p13.3;q13.1), inv(3)(q21.3q26.2), atau t(3;3)(q21.3;q26.2). DS-AMegL juga diklasifikasikan sendiri ke dalam Myeloid Leukemia associated Down Syndrome.3Prognosis AMegL pada pasien dewasa yang diobati jauh di bawah bentuk AMegL lainnya. Waktu kelangsungan hidup rata-rata hanya 18 hingga 41 minggu dengan tingkat kelangsungan hidup 5 tahun hanya 10-11 persen. Perbaikan besar dalam statistik ini kemungkinan akan membutuhkan pendekatan pengobatan baru yang diarahkan pada mekanisme yang mendasari penyakit ini.1

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Bataller, Alex, Ana Garrido, Francesca Guijarro, Guadalupe Oñate, Marina Diaz-Beyá, Montserrat Arnan, Mar Tormo et al. « European LeukemiaNet 2017 risk stratification for acute myeloid leukemia : validation in a risk-adapted protocol ». Blood Advances 6, n^o 4 (15 février 2022) : 1193–206. http://dx.doi.org/10.1182/bloodadvances.2021005585.

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Abstract The 2017 European LeukemiaNet (ELN 2017) guidelines for the diagnosis and management of acute myeloid leukemia (AML) have become fundamental guidelines to assess the prognosis and postremission therapy of patients. However, they have been retrospectively validated in few studies with patients included in different treatment protocols. We analyzed 861 patients included in the Cooperativo Para el Estudio y Tratamiento de las Leucemias Agudas y Mielodisplasias-12 risk-adapted protocol, which indicates cytarabine-based consolidation for patients allocated to the ELN 2017 favorable-risk group, whereas it recommends allogeneic stem cell transplantation (alloSCT) as a postremission strategy for the ELN 2017 intermediate- and adverse-risk groups. We retrospectively classified patients according to the ELN 2017, with 327 (48%), 109 (16%), and 245 (36%) patients allocated to the favorable-, intermediate-, and adverse-risk group, respectively. The 2- and 5-year overall survival (OS) rates were 77% and 70% for favorable-risk patients, 52% and 46% for intermediate-risk patients, and 33% and 23% for adverse-risk patients, respectively. Furthermore, we identified a subgroup of patients within the adverse group (inv(3)/t(3;3), complex karyotype, and/or TP53 mutation/17p abnormality) with a particularly poor outcome, with a 2-year OS of 15%. Our study validates the ELN 2017 risk stratification in a large cohort of patients treated with an ELN-2017 risk-adapted protocol based on alloSCT after remission for nonfavorable ELN subgroups and identifies a genetic subset with a very poor outcome that warrants investigation of novel strategies.

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Ruilova Gavilanes, Karen Michelle, Ari Estefano Jerves Crespo, Luis Andrés Bodero Acuña et Ana Gabriela Carrera Chulde. « Pioderma gangrenoso ». RECIAMUC 5, n^o 4 (20 novembre 2021) : 92–99. http://dx.doi.org/10.26820/reciamuc/5.(4).noviembre.2021.92-99.

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El pioderma gangrenoso (PG) es una dermatosis neutrofílica infrecuente, crónica, dolorosa y recidivante, idiopática en el 25-50% de los pacientes, con una presentación morfológica distintiva pero variable que abarca las formas pustulosas, ampollosa, ulcerativa y granulomatosa superficial o vegetante. De forma característica, en el 20-50% de las ocasiones aparece sobre piel previamente traumatizada; hasta en un 75% de los casos se asocia a una enfermedad sistémica previa, concurrente o posterior a su aparición, como la enfermedad inflamatoria intestinal (colitis ulcerativa, enfermedad de Crohn, 20-30%), poliartritis (20%), trastornos hematológicos (leucemia mieloide aguda y crónica, tricoleucemia, mielodisplasia, gammapatía monoclonal IgA) (15-25%), puede ser una manifestación cutánea de enfermedades autoinflamatorias monogénicas, como el síndrome PAPA (pyogenic arthritis, PG and acne), PASH (PG, acne and suppurative hidradenitis) o PAPASH (pyogenic arthritis, acne, PG and suppurative hidradenitis) o aparecer como acompañante de una dermatosis pustulosa subcórnea, enfermedad de Behçet o del síndrome de Sweet; raramente se debe a medicamentos. La metodología utilizada para el presente trabajo de investigación, se enfoca hacia una metodología orientada hacia la necesidad de indagar en forma precisa y coherente una situación. Enmarcada dentro de una revisión bibliográfica de tipo documental, ya que nos vamos a ocupar de temas planteados a nivel teórico como es Pioderma gangrenoso. La técnica para la recolección de datos está constituida por materiales impresos, audiovisuales y electrónicos, estos últimos como Google Académico, PubMed, entre otros. La información aquí obtenida será revisada para su posterior análisis. En base a los casos aquí presentados, se confirma lo analizado en la literatura revisada, que los casos de piodermas gangrenosos, se controlan con farmacología, específicamente con corticoesteroides, analgésicos y desinflamatorios, que en la mayoría de los casos evolucionan favorablemente. Otro punto a considerar es que también se prueba que otras patologías o comorbilidades que pueda tener un paciente, pueden desencadenar procedimientos que terminen en piodermas gangrenosos, en uno de los casos clínicos aquí presentados uno de los pacientes presento lesión ulcerada probablemente como consecuencia de la diabetes mellitus que padece desde hace mucho tiempo, en otros casos es posible la generación de esta patología como enfermedades de Crohn, leucemia, artritis reumatoide, entre otras.

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Caparros, Isabel, Regina Garcia-Delgado, Arturo Campos, Ana Rosell, Ma Paz Queipo de Llano, Santiago de la Torre, Gemma Ramirez, Laura Entrena, Eulogio Conde et Santiago Del Castillo. « The effect of Desferasirox in expression of Adhesion molecules in Neutrophils and Monocytes, in the Platelets Activacion and in the Atherothrombosis in Patients with Mielodisplastic Syndrome (MDS) and iron Overload. » Blood 114, n^o 22 (20 novembre 2009) : 1346. http://dx.doi.org/10.1182/blood.v114.22.1346.1346.

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Abstract Abstract 1346 Poster Board I-368 Introduction The interaction of leukocytes, platelets and vascular endothelial plays a fundamental role in the development of the arteriosclerotic injuries. This initial interaction is caused by P-selectin. This P-selectin is expressed on the surface of activated platelets and its recipient, ligand 1 of the glycoprotein P-selectin (PSGL-1) of the monocytes and granulocytes. In this interaction the microparticles derived from platelets, leukocytes and endothelial cells play a role fundamentally too. Platelets microparticles are formed by a phenomenon of vesiculation of the activated platelets by agonists as the thrombin, the collagen, among others. They induce the adherence of monocytes and granulocytes to endothelial. Besides, they amplify all signs that will give rise to an increase of the activity of adhesion molecules which will culminate in the expression of the tissue factor and its transformation into a procoagulant surface. As we know, the iron contributes to oxidative changes in tissues through the Fenton's reaction, which generates free radicals that would affect the activation of platelets through the reduction of types derived from the nitric acid. Objective To evaluate the effect of desferasirox in the activation of platelets and the development of atherothrombotic factors in patients with MDS and iron overload secondary to transfusions. Patients and methods 16 patients with MDS and iron overload secondary to transfusion, as well as 15 healthy controls were included. Chelation treatment was administered with 20 mg / kg / a day of desferasirox to 8 patients, 30 mg / kg / a day to 4 patients, 10 mg / kg / a day to 3 of them (2 for gastrointestinal toxicity and 1 for renal toxicity) and one withdrew the treatment due to ocular toxicity. They all received treatment at least one month. They were studied at the beginning of the treatment with desferasirox and after the treatment (ferritin 1000-1200), biomarkers of platelets activation (expression of anexin-V and P-selectin), the expression of adhesion molecules in monocytes and granulocytes (VCAM-1, ICAM-1, E-selectin, PSGL-1 and b1-integrin) and the percentage of interactions monocytes-platelets. Results The patients with MDS and iron overload presented basal values of platelets activation and of expression of adhesion molecules above the healthy controls. The patients treated with desferasirox that obtained a decrease in their ferritin figures (10/16) presented statistically significant reductions of the scoreboards of platelets activation with the consistent improvement of the membrane damage in the vascular endothelial. Conclusions It seems that a suitable chelating of the iron in the patients with MDS and iron overload reduces platelets activation and different parameters of activation in monocytes and granulocytes that would contribute to the tissue damage in the vascular endothelial. Other additional studies will be necessary to correlate these changes with the long-term morbidity, the mortality and quality of life in this population of patients. Disclosures No relevant conflicts of interest to declare.

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Kirwiastiny, Rina, Ringgo Alfarisi, Hidayat Hidayat et Ageel Al-Aziz Marjaen. « Hubungan Derajat Aktivitas Penyakit Lupus Eritematosus Sistemik Berdasarkan Skor Mex-Sledai Dengan Kejadian Anemia Pada Penderita Lupus Eritematosus Sistemik Di Komunitas Odapus Lampung ». Malahayati Nursing Journal 3, n^o 2 (5 février 2021) : 192–202. http://dx.doi.org/10.33024/manuju.v3i2.3596.

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ABSTRACT : RELATIONSHIP OF SYSTEMIC LUPUS ERYTHEMATOSUS ACTIVITIES BASED ON MEX-SLEDAI SCORE WITH INCIDENCE OF ANEMIA IN SYSTEMIC LUPUS ERYTHEMATOUS PATIENTS IN THE ODAPUS LAMPUNG COMMUNITY, 2020Background : Systemic Lupus Erytematosus (SLE) is a complex autoimmune disease characterized by the presence of autoantibodies against the cell nucleus and involving many organ systems in the body. Anemia in LES patients varies between chronic disease anemia, hemolytic anemia, blood loss, renal insufficiency, infection, myelodysplasia, and aplastic anemia. What often occurs in LES anemia is due to erythropoesis suppression due to chronic inflammation. Anemia in LES patients is an immune or non-immune disease. Anemia is a non-immune disease is anemia in chronic disease, iron deficiency anemia, sideroblastic anemia, anemia in kidney disease, anemia indicated by drugs, and anemia secondary to other diseases (eg sickle cell anemia).Research purposes : This study was to determine the degree of activity of systemic lupus erythematosus based on max-sledai and hemoglobin levels in systemic lupus erythematous patients in the ODAPUS Lampung community in 2020.Methode :The analytical observational method was used using a cross sectional approach. The research subjects were 30 respondents who used the total sampling technique from members of the ODAPUS Lampung community by conducting MEX-SLEDAI interviews and blood sampling conducted from November 2019 to February 2020. Statistical test used Fisher exact test.Results: From 30 study subjects, disease activity based on MEX-SLEDAI was above the average of 21 patients (70%). And the results of blood tests were 18 patients (60%) who were not anemia and 12 patients (40%) had anemia.Conclusion : There was a significant relationship between the degree of activity of Systemic Lupus Erythematosus based on the MEX-SLEDAI score and the incidence of anemia with p value = 0.024 meaning the p value ≤ 0.05. Keywords : LES; Incidence of Anemia; MEX-SLEDAI INTISARI : HUBUNGAN DERAJAT AKTIVITAS PENYAKIT LUPUS ERITEMATOSUS SISTEMIK BERDASARKAN SKOR MEXSLEDAI DENGAN KEJADIAN ANEMIA PADA PENDERITA LUPUS ERITEMATOUS SISTEMIK DI KOMUNITAS ODAPUS LAMPUNG Latar belakang : Systemic Lupus Erytematosus (SLE) merupakan penyakit autoimun yang kompleks ditandai oleh adanya autoantibodi terhadap inti sel dan melibatkan banyak sistem organ dalam tubuh. Anemia pada pasien LES bervariasi antara anemia penyakit kronis, anemia hemolitik, kehilangan darah, insufisiensi ginjal, infeksi, mielodisplasia, dan anemia aplastik. Yang sering terjadi anemia pada LES disebabkan supresi eritropoesis karena inflamasi yang kronis. Anemia pada pasien LES merupakan penyakit imun atau non-imun. Anemia merupakan penyakit non-imun adalah anemia pada penyakit kronik ,anemia defisiensi besi, anemia sideroblastik, anemia pada penyakit ginjal, anemia indikasi obat, dan anemia sekunder terhadap penyakit lain ( misalnya anemia sel sabit ).Tujuan Penelitian : Penelitian ini untuk mengetahui hubungan drajat aktivitas penyakit lupus eritematosus sistemik berdasarkan max-sledai dengan kadar hemoglobin pada penderita lupus eritematous sistemik di komunitas ODAPUS lampung tahun 2020.Metode : Digunakan metode observasional analitik menggunakan pendekatan cross sectional. Subjek penelitian sebanyak 30 responden yang menggunakan teknik total sampling dari anggota komunitas ODAPUS Lampung dengan melakukan wawancara MEX-SLEDAI dan pengambilan sampel darah yang dilakukan pada bulan November 2019 s/d Februari 2020. Uji statistic menggunakan Fisher exact test.Hasil : Dari 30 subjek penelitian didapatkan aktifitas penyakit berdasarkan MEX-SLEDAI di atas rata – rata sebanyak 21 pasien (70%). Dan hasil peneriksaan darah yaitu 18 pasien (60%) yang Tidak anemia dan yang mengalami Anemia ada 12 pasien (40%).Kesimpulan : Terdapat hubungan bermakna antara derajat aktivitas penyakit Lupus Eritematosus Sistemik berdasarkan skor MEX-SLEDAI dengan Kejadian Anemia dengan p value =0.024 berarti nilai p value ≤ 0.05. Kata Kunci : LES; Kejadian Anemia; MEX-SLEDAI

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Kirwiastiny, Rina, Ringgo Alfarisi, Hidayat Hidayat et Ageel Al-Aziz Marjaen. « Hubungan Derajat Aktivitas Penyakit Lupus Eritematosus Sistemik Berdasarkan Skor Mex-Sledai Dengan Kejadian Anemia Pada Penderita Lupus Eritematosus Sistemik Di Komunitas Odapus Lampung ». Malahayati Nursing Journal 3, n^o 2 (15 février 2021) : 192–202. http://dx.doi.org/10.33024/mnj.v3i2.3596.

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ABSTRACT : RELATIONSHIP OF SYSTEMIC LUPUS ERYTHEMATOSUS ACTIVITIES BASED ON MEX-SLEDAI SCORE WITH INCIDENCE OF ANEMIA IN SYSTEMIC LUPUS ERYTHEMATOUS PATIENTS IN THE ODAPUS LAMPUNG COMMUNITY, 2020Background : Systemic Lupus Erytematosus (SLE) is a complex autoimmune disease characterized by the presence of autoantibodies against the cell nucleus and involving many organ systems in the body. Anemia in LES patients varies between chronic disease anemia, hemolytic anemia, blood loss, renal insufficiency, infection, myelodysplasia, and aplastic anemia. What often occurs in LES anemia is due to erythropoesis suppression due to chronic inflammation. Anemia in LES patients is an immune or non-immune disease. Anemia is a non-immune disease is anemia in chronic disease, iron deficiency anemia, sideroblastic anemia, anemia in kidney disease, anemia indicated by drugs, and anemia secondary to other diseases (eg sickle cell anemia).Research purposes : This study was to determine the degree of activity of systemic lupus erythematosus based on max-sledai and hemoglobin levels in systemic lupus erythematous patients in the ODAPUS Lampung community in 2020.Methode :The analytical observational method was used using a cross sectional approach. The research subjects were 30 respondents who used the total sampling technique from members of the ODAPUS Lampung community by conducting MEX-SLEDAI interviews and blood sampling conducted from November 2019 to February 2020. Statistical test used Fisher exact test.Results: From 30 study subjects, disease activity based on MEX-SLEDAI was above the average of 21 patients (70%). And the results of blood tests were 18 patients (60%) who were not anemia and 12 patients (40%) had anemia.Conclusion : There was a significant relationship between the degree of activity of Systemic Lupus Erythematosus based on the MEX-SLEDAI score and the incidence of anemia with p value = 0.024 meaning the p value ≤ 0.05. Keywords : LES; Incidence of Anemia; MEX-SLEDAI INTISARI : HUBUNGAN DERAJAT AKTIVITAS PENYAKIT LUPUS ERITEMATOSUS SISTEMIK BERDASARKAN SKOR MEXSLEDAI DENGAN KEJADIAN ANEMIA PADA PENDERITA LUPUS ERITEMATOUS SISTEMIK DI KOMUNITAS ODAPUS LAMPUNG Latar belakang : Systemic Lupus Erytematosus (SLE) merupakan penyakit autoimun yang kompleks ditandai oleh adanya autoantibodi terhadap inti sel dan melibatkan banyak sistem organ dalam tubuh. Anemia pada pasien LES bervariasi antara anemia penyakit kronis, anemia hemolitik, kehilangan darah, insufisiensi ginjal, infeksi, mielodisplasia, dan anemia aplastik. Yang sering terjadi anemia pada LES disebabkan supresi eritropoesis karena inflamasi yang kronis. Anemia pada pasien LES merupakan penyakit imun atau non-imun. Anemia merupakan penyakit non-imun adalah anemia pada penyakit kronik ,anemia defisiensi besi, anemia sideroblastik, anemia pada penyakit ginjal, anemia indikasi obat, dan anemia sekunder terhadap penyakit lain ( misalnya anemia sel sabit ).Tujuan Penelitian : Penelitian ini untuk mengetahui hubungan drajat aktivitas penyakit lupus eritematosus sistemik berdasarkan max-sledai dengan kadar hemoglobin pada penderita lupus eritematous sistemik di komunitas ODAPUS lampung tahun 2020.Metode : Digunakan metode observasional analitik menggunakan pendekatan cross sectional. Subjek penelitian sebanyak 30 responden yang menggunakan teknik total sampling dari anggota komunitas ODAPUS Lampung dengan melakukan wawancara MEX-SLEDAI dan pengambilan sampel darah yang dilakukan pada bulan November 2019 s/d Februari 2020. Uji statistic menggunakan Fisher exact test.Hasil : Dari 30 subjek penelitian didapatkan aktifitas penyakit berdasarkan MEX-SLEDAI di atas rata – rata sebanyak 21 pasien (70%). Dan hasil peneriksaan darah yaitu 18 pasien (60%) yang Tidak anemia dan yang mengalami Anemia ada 12 pasien (40%).Kesimpulan : Terdapat hubungan bermakna antara derajat aktivitas penyakit Lupus Eritematosus Sistemik berdasarkan skor MEX-SLEDAI dengan Kejadian Anemia dengan p value =0.024 berarti nilai p value ≤ 0.05. Kata Kunci : LES; Kejadian Anemia; MEX-SLEDAI

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Granjeiro, Claudia da Fonseca, Alex Semenoff-Segundo, Alessandra Nogueira Porto, Natalino Francisco da Silva, Álvaro Henrique Borges et Tereza Aparecida Delle Vedove Semenoff. « Epidemiological Profile of Patients with Hematological Neoplasms in an Oncological Hospital of Mato Grosso ». Journal of Health Sciences 20, n^o 4 (30 décembre 2018) : 232. http://dx.doi.org/10.17921/2447-8938.2018v20n4p232-237.

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O objetivo do estudo foi analisar os aspectos epidemiológicos de pacientes adultos com diagnóstico de neoplasia hematológica, no período de 2004 a 2014, em um Hospital Oncológico em Mato Grosso. A coleta de dados foi realizada através de busca e análise de 590 prontuários. Entre os pacientes, 335 (56,8%) eram do sexo masculino (p<0,05). A maioria possuía mais de 50 anos (p<0,05), com idade média de 53,97±16,55 anos. Quanto ao local de procedência, 257 pacientes eram provenientes da região metropolitana de Cuiabá (43,6%) e 333 (56,4%) de outros locais (p<0,05). A maioria eram pacientes não brancos (p<0,05) e solteiros 342 (58%) (p<0,05). Em relação ao tratamento, 537 (91%) pacientes utilizavam quimioterapia (p<0,05), 57 (9,7%) fizeram uso da radioterapia (p<0,05). Cinquenta e quatro (9,2%) pacientes fizeram uso de ambas as terapias de forma associada (p<0,05) e 50 pacientes (8,4%) não fizeram uso de radioterapia e nem de quimioterapia. Referente aos tipos de neoplasia, os diagnósticos mais encontrados na análise foram de: leucemia mielóide crônica 131 (22,2%); linfoma não Hodking 119 (20,2%); mieloma múltiplo 93 (15,8%); trombocitemia essencial 49 (8,3%); linfoma de Hodking 39 (6,6%); leucemia linfocítica crônica 38 (6,4%); policetemia vera 34 (5,8%); leucemia mielóide aguda 27 (4,6%); síndrome mielodisplasica 21 (3,6); leucemia linfocítica aguda 13 (2,2%) e síndrome mieloproliferativa 10 (1,7%) (p<0,05). A partir dos resultados deste estudo, pode-se concluir que, em geral, os pacientes mais acometidos pelas neoplasias hematológicas tinham mais de 50 anos, eram homens, provenientes do interior do Estado, não brancos e solteiros.Palavras-chave: Doenças Hematológicas. Epidemiologia. Leucemia.AbstractThe objective of this study was to analyze the epidemiological aspects of adult patients diagnosed with hematologic neoplasia, from 2004 to 2014, at a Cancer Hospital in Mato Grosso. Data collection was performed through the search and analysis of 590 patient records. Among the patients, 335 (56.8%) were male (p <0.05). The majority was older than 50 years (p <0.05), with a mean age of 53.97 ± 14.95 years. Regarding origin, 257 patients came from the metropolitan region of Cuiabá (43.6%) and 333 (56.4%) from other sites (p <0.05). The majority was non-white patients (p <0.05) and unmarried 342 (58%) (p <0.05). About treatment, 537 (91%) patients used chemotherapy (p <0.05), 57 (9.7%) used radiotherapy (p <0.05). Fifty-four (9.2%) patients used both therapies in an associated way (p <0.05) and 50 patients (8.4%) did not use either radiotherapy or chemotherapy at any time of their treatment. Regarding the types of neoplasia, the diagnoses most found in the analysis were: chronic myelogenous leukemia 131 (22.2%); non Hodking lymphoma 119 (20.2%); multiple myeloma 93 (15.8%); essential thrombocythemia 49 (8.3%); Hodgkin's lymphoma 39 (6.6%); chronic lymphocytic leukemia 38 (6.4%); policetemia vera 34 (5.8%); acute myeloid leukemia 27 (4.6%); myelodysplastic syndrome 21 (3,6); acute lymphocytic leukemia 13 (2.2%) and myeloproliferative syndrome 10 (1.7%) (p <0.05). From the results of this study, it can be concluded that, in general, the most affected patients by hematological malignancies were older than 50 years, were men from the interior of the State, non-white and unmarried.Keywords: Hematologic Diseases. Epidemiology. Hodgkin Lymphoma. Leukemia.

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Parma, Matteo, Elisa Doni, Federica Colnaghi, Arianna Colombo, Elena Elli, Pietro Pioltelli, Elisabetta Terruzzi et Enrico Maria Pogliani. « Chimerism Status Analysis after Hematopoietic Stem CellTransplantation on Granulocytes and Mononucleated Cells of Peripheral Blood ». Blood 112, n^o 11 (16 novembre 2008) : 4604. http://dx.doi.org/10.1182/blood.v112.11.4604.4604.

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Abstract Background Chimerism Status (CS) analysis is largely useful for evaluation of donor (D) cells engraftment after allogeneic haematopoietic stem cell transplantation (HSCT). The largest performed method is based on the evaluation of the DNA sequences “Short Tandem Repeats” (STR) and Variable Number Tandem Repeats” (VNTR). Their high polymorphism permits to differentiate donor (D) or recipient (R) origin of the analysed cells. PCR amplification and DNA sequencing permit to quantify D and R amount. Patients and methods CS analysis after HSCT can be conducted on different cell populations in marrow or peripheral blood: whole blood cells, mononucleated cells, or single separated populations. A complete CS (CCS) usually correlates with a favourable outcome of HSCT, conversely a mixed CS may predicts a relapse of disease or a rejection of HSCT, particularly in the condition defined as increasing-mixed CS (IMCS), in which the amount of R origin cells increases along the time. In our study we employed a simple and easily reproducible method to analyse CS on peripheral blood cells at +30, +60, +90 and +120 days after HSCT. CS analysis was conducted separately on granulocytes and mononucleated cells, obtained after double gradient centrifugation. A semi-quantitative method, based on multiplex PCR amplification of 16 STR markers using a commercial kit (PowerPlex® 16 System–Promega), was applied to define the difference between D and R. We assumed the CS in granulocyte population as expression of myeloid engraftment. We also correlated the CS in mononucleated population, with the different leukocyte subpopulations (lymphocytes, monocytes) in peripheral blood count and with the reconstitution of lymphocyte subpopulations (B, T cells and NK cells) at the same time point of analysis, in order to compare the reciprocal course. We evaluated 13 HSCT on 12 patients (pts) (1 pts was submitted to double HSCT for a rejection occurred after the first HSCT), 7 from sibling related donor (SRD) and 6 from matched unrelated donor (MUD). All the pts were affected by neoplastic disease in first (9) or next (3) remission: acute lymphoblastic (5) or myeloblastic leukaemia (4), mielodisplasia (1), chronic myeloid leukaemia (1), multiple myeloma (1). All the conditioning regimens were mieloablative, all the MUD-HSCT and one of the SRD-HSCT received Anti-Thymocyte-Globulin (ATG), 7/8 SRD-HSCT did not received ATG nor other T-depletion procedures. Results: Evaluation on time + 30, +60, +90 and +120 days after HSCT was performed on 13 HSCT, except in two pts who stopped before (+30 and +60) due to their death for relapse or toxicity. A stable CCS in granulocytes was observed in 10/13 HSCT since the first control. 3/13 HSCT presented IMCS followed by relapse (2 pts) or rejection. In mononucleated cells population 10/13 HSCT presented CCS at the first control, 2/10 evolved to CCS in the successive times. The 3 pts who relapsed or rejected presented a IMCS in mononucleated cells too. Considering the different leucocyte populations we observed that the mononucleate cells amount was constituted for only 50% from lymphocytes, the remaining 50% was represented from monocytes. Therefore the analysis of lymphocyte subpopulations showed that T-lymphocytes were virtually absent (either T4 or T8) till to +120 in pts who received ATG, so in these ones CS analysis in mononucleated cells was performed only in monocytes, B lymphocytes and NK cells. Instead pts who did not received ATG presented complete and more rapid reconstitution of all the lymphocyte subpopulations (median at +60 day: T4 200, T8 120, B 60, NK160 × 10^9/l). Conclusions: our method is simple and rapid in analysing CS. Granulocytes CS reflect the engraftment of myeloid lineage. Instead CS in mononucleated cells must be considered in the context of the type of transplantation (MUD or SRD, T-depletion or T repletion) and correlated with the single subpopulations of peripheral blood.

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Fagundes, Evandro M. « Tratamento do paciente com mielodisplasia de alto risco ». Revista Brasileira de Hematologia e Hemoterapia 28, n^o 3 (septembre 2006). http://dx.doi.org/10.1590/s1516-84842006000300014.

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OYARCE L, VERÓNICA, NATALIE RODRÍGUEZ Z, JUAN TORDECILLA C et PATRICIA VERDUGO L. « Mielodisplasia en Pacientes Pediátricos : Evaluación de Clasificaciones Actuales ». Revista chilena de pediatría 80, n^o 4 (août 2009). http://dx.doi.org/10.4067/s0370-41062009000400005.

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MUÑOZ M, MARCELA, NATALIE RODRÍGUEZ Z, JUAN TORDECILLA C, ESTER URETA H, CARLOS RIZZARDINI L, VERÓNICA SOTO A et KARIN WENSIOE R. « "Peliosis Hepatis" Como Complicación del Uso de Anticonceptivos Orales en una Paciente con Mielodisplasia ». Revista chilena de pediatría 80, n^o 4 (août 2009). http://dx.doi.org/10.4067/s0370-41062009000400007.

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Olave Rubio, M. T., T. Iturbe Hernández, A. Arruga Manzano, J. C. Sola Lapeña et L. Palomera Bernal. « Mielodisplasia con deleción del cromosoma 7 asociada a tuberculosis y hepatitis C : ¿un modelo de sinergismo inmunodepresor ? » Anales de Medicina Interna 18, n^o 12 (décembre 2001). http://dx.doi.org/10.4321/s0212-71992001001200012.

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Medina Medina, Elcy Lucía, Beatriz Molinares Arévalo, Rafael Cárdenas Restrepo, Ana María Valencia Cadavid, Daniel Fernando Arias Betancurt et José William Cornejo Ochoa. « Aspectos imaginológicos del sarcoma granulocítico en la cara y el cráneo de niños y jóvenes : informe de cinco casos y revisión de la literatura ». Iatreia 23, n^o 3 (6 décembre 2012). http://dx.doi.org/10.17533/udea.iatreia.13926.

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Introducción: el sarcoma granulocítico (SG) es una neoplasia maligna cuya incidencia es de 2,9% a 3,1% en pacientes con leucemia mieloide (LM) o enfermedades mieloproliferativas. Se presenta habitualmente en hombres y en población africana, asiática y suramericana.Objetivo: describir las características imaginológicas del SG en la cara y el cráneo de cinco niños y jóvenes.Pacientes y métodos: presentamos cinco pacientes con SG en la cara y la base del cráneo. A cuatro de ellos se les hizo tomografía computarizada (TC) y a tres, resonancia magnética (RM) cerebral.Cuatro presentaron una masa de tejido blando en la órbita, dos tenían afectación ósea y otro reveló lesión en el sistema nervioso central. En cuatro se diagnosticó leucemia mieloide aguda (LMA).Conclusión: el SG puede manifestarse con invasión orbitaria y craneofacial en niños y adultos jóvenes. Usualmente los pacientes consultan por proptosis y edema orbitario. Con este cuadro clínico el SG es la primera probabilidad diagnóstica en el contexto de la LMA o las mielodisplasias. En otras situaciones clínicas se debe hacer diagnóstico diferencial con complicaciones de sinusitis, rabdomiosarcoma, linfoma de la órbita y otras neoplasias. La imaginología demuestra invasión de tejidos blandos e infiltración ósea. Es muy característico del SG afectar en un comienzo la pared lateral o la superior de la órbita. En algunos casos simula abscesos. El diagnóstico se confirma por histopatología.

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