Journal articles: 'Sickle cell pathology'

1

Malowany, Janet I., and Jagdish Butany. "Pathology of sickle cell disease." Seminars in Diagnostic Pathology 29, no. 1 (February 2012): 49–55. http://dx.doi.org/10.1053/j.semdp.2011.07.005.

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2

Rice-Evans, C., S. C. Omorphos, and E. Baysal. "Sickle cell membranes and oxidative damage." Biochemical Journal 237, no. 1 (July 1, 1986): 265–69. http://dx.doi.org/10.1042/bj2370265.

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Sickle erythrocytes and their membranes are susceptible to endogenous free-radical-mediated oxidative damage which correlates with the proportion of irreversibly sickled cells. The suppression of incubation-induced oxidative stress by antioxidants, free radical scavengers and an iron chelator suggest that oxidation products of membrane-bound haemoglobin contribute towards the pathology of the disease.

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&NA;. "Liver Pathology In Sickle Cell Diseases." Advances in Anatomic Pathology 2, no. 5 (September 1995): 336. http://dx.doi.org/10.1097/00125480-199509000-00009.

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Diwan, B. A., M. T. Gladwin, C. T. Noguchi, J. M. Ward, A. L. Fitzhugh, and G. S. Buzard. "Renal Pathology in Hemizygous Sickle Cell Mice." Toxicologic Pathology 30, no. 2 (February 2002): 254–62. http://dx.doi.org/10.1080/019262302753559597.

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5

Hawley, Dean A., and Leo J. McCarthy. "Sickle Cell Disease." American Journal of Forensic Medicine and Pathology 30, no. 1 (March 2009): 69–71. http://dx.doi.org/10.1097/paf.0b013e3181873c90.

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6

Manci, E. A. "Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease." Blood 107, no. 4 (February 15, 2006): 1651–58. http://dx.doi.org/10.1182/blood-2005-07-2839.

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7

Sheshanna, Nanditha, Gurpreet Sethi, and Sumitha M. Prakash. "Sudden Death in Sickle Cell Disease: An Autopsy Diagnosis." Journal of Medical Sciences 3, no. 4 (2017): 113–15. http://dx.doi.org/10.5005/jp-journals-10045-0069.

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ABSTRACT Sickle cell disease is a common hereditary hemoglobinopathy with high prevalence in the central and northeastern regions of India. A 24-year-old male patient with 3 days’ history of fever was brought dead to the hospital. Morphology showed clogging of blood vessels with sickled red blood cells (RBCs) in all the organs, and an autopsy diagnosis of sickle cell disease was made. As the cause may not be obvious in many cases, most patients remain undiagnosed. It is important to note the circumstances of death, gross finding, and histopathology, with hemoglobin electrophoresis if available, during autopsy to arrive at the diagnosis. This case is presented here to highlight this fact and draw attention to its pathology. How to cite this article Sheshanna N, Sethi G, Raj JA, Prakash SM, Surhonne SP. Sudden Death in Sickle Cell Disease: An Autopsy Diagnosis. J Med Sci 2017;3(4):113-115.

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Gheorghe, Gabriela, Marian Rollins-Raval, and Miguel Reyes-Múgica. "Sickle Cell Disease." International Journal of Surgical Pathology 17, no. 3 (December 8, 2008): 270–71. http://dx.doi.org/10.1177/1066896908328578.

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9

Jacobs, A. "Sickle Cell Disease." Journal of Clinical Pathology 39, no. 7 (July 1, 1986): 815. http://dx.doi.org/10.1136/jcp.39.7.815-a.

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10

Rudy, Hayeem L., David Yang, Andrew D. Nam, and Woojin Cho. "Review of Sickle Cell Disease and Spinal Pathology." Global Spine Journal 9, no. 7 (September 17, 2018): 761–66. http://dx.doi.org/10.1177/2192568218799074.

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Study Design:Sickle cell disease (SCD) is a relatively common blood disorder that has profound implications on the musculoskeletal system and particularly the spine; however, there is a paucity of data in the literature discussing this important topic.Objectives:(1) To elucidate common spinal pathologies affecting patients with SCD, as well as the medical and surgical treatments available for these patients. (2) To discuss indications for surgical management of spinal complications of SCD and important for orthopedic surgeons when taking patients with SCD to the operating room.Methods:A narrative review of the literature was performed.Results:Patients with SCD have a significantly higher risk of developing spinal pathologies including vertebral osteomyelitis, compression fracture, vertebral vaso-occlusive crises, and osteoporosis, among others. In addition, patients with sickle cell disease are particularly susceptible to developing perioperative and post-operative complications including surgical site infection, implant malfunction, and vertebral body compression fracture. Postoperatively patients with SCD are prone to developing complications and adequate hydration is necessary in order to reduce complications of SCD.Conclusions:Several spinal pathologies may arise secondary to SCD and distinguishing these pathologies from one another may be challenging due to similarities in symptoms and inflammatory markers. Although most patients with SCD can benefit from conservative treatment involving rest, symptomatic therapy, antibiotic therapy, and/or orthosis, surgical intervention may be indicated in certain cases. It is preferable to avoid surgery in patients with SCD due to an increased risk of complications such as wound infection and vaso-occlusive crisis.

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Sadler, Katelyn E., Tylor R. Lewis, Tyler B. Waltz, Joseph C. Besharse, and Cheryl L. Stucky. "Peripheral nerve pathology in sickle cell disease mice." PAIN Reports 4, no. 4 (2019): e765. http://dx.doi.org/10.1097/pr9.0000000000000765.

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12

Lusco, Mark A., Agnes B. Fogo, Behzad Najafian, and Charles E. Alpers. "AJKD Atlas of Renal Pathology: Sickle Cell Nephropathy." American Journal of Kidney Diseases 68, no. 1 (July 2016): e1-e3. http://dx.doi.org/10.1053/j.ajkd.2016.05.003.

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13

Lukwete, Nadine Keleku, Mikiko Suzuki, Harit Panda, Akihito Otsuki, Fumiki Katsuoka, Ritsumi Saitou, Daisuke Saigusa, Akira Uruno, and Masayuki Yamamoto. "How KEAP1 Deletion in Single Cell Mitigates Sickle Cell Pathology." Blood 132, Supplement 1 (November 29, 2018): 2353. http://dx.doi.org/10.1182/blood-2018-99-110502.

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Abstract Sickle cell disease (SCD) is one of the most frequent blood disorders caused by a point mutation of β-globin gene. In the pathology of SCD, we often encounter contributions of vascular and cellular components. The activation of vascular endothelial cells promotes adhesion of inflammatory cells and vaso-occlusion responsible for low blood flow, hypoxemia and organ damages. Hemolysis triggers activation and recruitment of myeloid cells to the inflammation site, further sustaining the inflammatory process. Recent progress has created the need to examine targeted induction of the KEAP1-NRF2 system as a potential therapeutic tool for the SCD. We have demonstrated that genetic as well as pharmacological induction of NRF2 in SCD prevent organ damages and suppress inflammation, although it still remains unclear how the NRF2 induction modifies the pathology or which type of cells contribute to the NRF2-mediated improvement of SCD. To address these questions, we conducted targeted deletion of Keap1 gene and induced expression of Nrf2 gene in endothelial cells and macrophages/monocytes using Tie1-Cre and LysM-Cre system, respectively. We found that SCD::Keap1F/F::Tie1-Cre mice (NRF2-increase in endothelial cells) showed low hematocrit and low platelet counts. While severe infiltration of monocytes, lymphocytes and macrophages was observed in the lungs of SCD mice, such phenotype was diminished in the SCD::Keap1F/F::Tie1-Cre mice. In the compound mutant mice, livers showed fewer necrotic zones and less to none accumulation of free iron. On the contrary, imaging MS (iMScope) analyses revealed decrease of free heme in livers and spleens in SCD::Keap1F/F::LysM-Cre mice (NRF2-increase in macrophages/monocytes). Furthermore, upregulations of most known Nrf2-dependent pathways and other yet-to-be-defined pathways are found in an RNA-sequencing analysis of the mice. Importantly, NRF2 induction enhanced heme catabolism and decreases the plasma level of free heme and the quantity of accumulated free heme in the SCD::Keap1F/F::LysM-Cre mouse tissues, whereas the change is not obvious in SCD::Keap1F/F::Tie1-Cre mice. On the contrary, vascular leakage of fluids was decreased in the SCD::Keap1F/F::Tie1-Cre mice. Thus, our results demonstrate that cell-lineage-specific activation of Nrf2 does inhibit the inflammation and organ injury with some particularities to each genotype. Disclosures No relevant conflicts of interest to declare.

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Bandyopadhyay, Ranjana, SanjayK Bandyopadhyay, and Anita Dutta. "Sickle cell hepatopathy." Indian Journal of Pathology and Microbiology 51, no. 2 (2008): 284. http://dx.doi.org/10.4103/0377-4929.41698.

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15

Lew, Virgilio L., and Robert M. Bookchin. "Ion Transport Pathology in the Mechanism of Sickle Cell Dehydration." Physiological Reviews 85, no. 1 (January 2005): 179–200. http://dx.doi.org/10.1152/physrev.00052.2003.

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Polymers of deoxyhemoglobin S deform sickle cell anemia red blood cells into sickle shapes, leading to the formation of dense, dehydrated red blood cells with a markedly shortened life-span. Nearly four decades of intense research in many laboratories has led to a mechanistic understanding of the complex events leading from sickling-induced permeabilization of the red cell membrane to small cations, to the generation of the heterogeneity of age and hydration condition of circulating sickle cells. This review follows chronologically the major experimental findings and the evolution of guiding ideas for research in this field. Predictions derived from mathematical models of red cell and reticulocyte homeostasis led to the formulation of an alternative to prevailing gradualist views: a multitrack dehydration model based on interactive influences between the red cell anion exchanger and two K+transporters, the Gardos channel (hSK4, hIK1) and the K-Cl cotransporter (KCC), with differential effects dependent on red cell age and variability of KCC expression among reticulocytes. The experimental tests of the model predictions and the amply supportive results are discussed. The review concludes with a brief survey of the therapeutic strategies aimed at preventing sickle cell dehydration and with an analysis of the main open questions in the field.

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16

Wu, Li-Chen, Chiao-Wang Sun, Thomas M. Ryan, Kevin M. Pawlik, Jinxiang Ren, and Tim M. Townes. "Correction of sickle cell disease by homologous recombination in embryonic stem cells." Blood 108, no. 4 (August 15, 2006): 1183–88. http://dx.doi.org/10.1182/blood-2006-02-004812.

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Abstract Previous studies have demonstrated that sickle cell disease (SCD) can be corrected in mouse models by transduction of hematopoietic stem cells with lentiviral vectors containing antisickling globin genes followed by transplantation of these cells into syngeneic recipients. Although self-inactivating (SIN) lentiviral vectors with or without insulator elements should provide a safe and effective treatment in humans, some concerns about insertional mutagenesis persist. An ideal correction would involve replacement of the sickle globin gene (βS) with a normal copy of the gene (βA). We recently derived embryonic stem (ES) cells from a novel knock-in mouse model of SCD and tested a protocol for correcting the sickle mutation by homologous recombination. In this paper, we demonstrate the replacement of the human βS-globin gene with a human βA-globin gene and the derivation of mice from these cells. The animals produce high levels of normal human hemoglobin (HbA) and the pathology associated with SCD is corrected. Hematologic values are restored to normal levels and organ pathology is ameliorated. These experiments provide a foundation for similar studies in human ES cells derived from sickle cell patients. Although efficient methods for production of human ES cells by somatic nuclear transfer must be developed, the data in this paper demonstrate that sickle cell disease can be corrected without the risk of insertional mutagenesis.

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17

Kwun Lui, Shu, Alyssa Krasinskas, Rushikesh Shah, and Jessica M. Tracht. "Orthotropic Liver Transplantation for Acute Intrahepatic Cholestasis in Sickle Cell Disease: Clinical and Histopathologic Features of a Rare Case." International Journal of Surgical Pathology 27, no. 4 (September 10, 2018): 411–17. http://dx.doi.org/10.1177/1066896918798467.

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Sickle cell disease has a wide range of hepatic manifestations, with acute intrahepatic cholestasis being one of the rarest and most fatal, often resulting in acute fulminant hepatic failure. Liver transplantation is an emerging but rarely utilized treatment for hepatic failure in the setting of sickle cell disease. Few such cases have been reported in the literature, with little emphasis on histopathologic correlation. We report a case of acute intrahepatic cholestasis in a patient with sickle cell disease who underwent orthotropic liver transplantation and describe novel correlating histopathologic features. The patient is a 29-year-old man who presented with hyperbilirubinemia, acute kidney injury, and coagulopathy. He was diagnosed clinically with acute intrahepatic cholestasis and received an orthotropic liver transplant. The explanted liver demonstrated marked sinusoidal expansion by sickled erythrocytes, hyperplastic Kupffer cells, and extramedullary hematopoiesis. There was extensive sinusoidal and centrizonal fibrosis with occlusion of central veins reminiscent of chronic sinusoidal obstructive syndrome, a previously undescribed pattern of injury. This case represents one of the few reported cases of sickle cell intrahepatic cholestasis treated by transplantation and demonstrates the rarely reported histopathologic features and gives insight to a potentially new mechanism of injury in these patients. Familiarity with the morphologic features of sickle cell hepatopathy and its clinical manifestations is important as transplantation in sickle cell–related liver injury increases in frequency.

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18

Dudley, Alden W., and Caroline C. Waddell. "Crisis in sickle cell trait." Human Pathology 22, no. 6 (June 1991): 616–18. http://dx.doi.org/10.1016/0046-8177(91)90242-h.

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19

Kim-Shapiro, Daniel B., and Mark T. Gladwin. "Nitric oxide pathology and therapeutics in sickle cell disease." Clinical Hemorheology and Microcirculation 68, no. 2-3 (March 28, 2018): 223–37. http://dx.doi.org/10.3233/ch-189009.

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20

Barman, Charles R., Angelo Collins, Edward J. Louis, and John R. Jungck. "Sickle Cell Anemia: "Interesting Pathology" and "Rarely Told Stories"." American Biology Teacher 47, no. 3 (March 1, 1985): 183–87. http://dx.doi.org/10.2307/4448004.

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21

Al-Humood, Salah, Rajaa Zueriq, Lama Al-Faris, Rajaa Marouf, and Fahd Al-Mulla. "Circulating Cell-Free DNA in Sickle Cell Disease: Is It a Potentially Useful Biomarker?" Archives of Pathology & Laboratory Medicine 138, no. 5 (May 1, 2014): 678–83. http://dx.doi.org/10.5858/arpa.2012-0725-oa.

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Context.—Vascular occlusion in sickle cell disease causes increased levels of plasma cell-free DNA as a result of cell death and tissue damage. Objectives.—This study investigates plasma cell-free DNA concentrations in sickle cell disease patients, and aims at exploring the significance of plasma cell-free DNA as a potential biomarker in predicting its complications. Design.—Plasma cell-free DNA levels were measured using real-time quantitative polymerase chain reaction to quantitatively measure β-globin gene in blood samples from 57 sickle cell disease patients with acute vaso-occlusive crisis, 42 patients in steady state, 16 individuals with sickle cell trait, and 40 healthy controls. Results.—Plasma cell-free DNA level was significantly elevated in samples from patients with acute vaso-occlusive crisis when compared with those in steady state (P = .002), and was significantly higher both in crisis and in steady state when compared with individuals with sickle cell trait and healthy controls (P < .001). There was no difference in cell-free DNA levels between individuals with sickle cell trait and healthy controls. There was no association between plasma cell-free DNA levels and various clinical complications of sickle cell disease and comorbidity. Conclusions.—Plasma cell-free DNA, as quantified by polymerase chain reaction amplification of the β-globin and human telomerase reverse transcriptase genes, is increased in sickle cell disease patients in vaso-occlusive crisis and in steady state compared with individuals with sickle cell trait and healthy controls, and may be used as a tool to diagnose and monitor the sickle cell crisis and differentiate post–packed red cell transfusion sickle cell disease patients from individuals with sickle cell trait.

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22

Sparkenbaugh, Erica M., Pichika Chantrathammachart, Jacqueline Mickelson, Joanne van Ryn, Robert P. Hebbel, Dougald M. Monroe, Nigel Mackman, Nigel S. Key, and Rafal Pawlinski. "Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease." Blood 123, no. 11 (March 13, 2014): 1747–56. http://dx.doi.org/10.1182/blood-2013-08-523936.

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Key Points Inhibition of FXa or thrombin might be considered to reduce thrombotic complications and vascular inflammation in sickle cell patients. PAR-2 could be a potential target to inhibit vascular pathology associated with sickle cell disease.

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23

Weber, Marc L., Robert P. Hebbel, and Kalpna Gupta. "Morphine Induces Kidney Injury in Transgenic Sickle Cell Mice." Blood 106, no. 11 (November 16, 2005): 3167. http://dx.doi.org/10.1182/blood.v106.11.3167.3167.

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Abstract Sickle cell nephropathy is poorly understood and currently lacks any specific therapeutic options. Opioids are used to treat the pain associated with sickle cell disease. Interestingly, heroin (a metabolic precursor of morphine) is associated with glomerulosclerosis. Glomerulomegaly, an early sign of kidney damage, predicts the eventual severity of glomerulosclerosis. Using transgenic sickle mice as a disease model, we evaluated the effects of chronic opioid exposure on kidney pathology in the setting of sickle cell disease. Transgenic sickle cell mice (carry a single copy of linked transgenes for human alpha and beta-S globins and are homozygous for deletion of murine beta major globin) were treated for three weeks with PBS, morphine (50–150mg/70kg human equivalent), naloxone (equimolar to morphine), or morphine and naloxone (n=6 per group). We evaluated the effects of morphine on kidney pathology by assessing kidney weight, glomerular volume (by the Weibel-Gomez method), and mesangial cell counts per glomerulus. Morphine stimulated kidney growth in transgenic sickle mice. Treatment with morphine significantly increased glomerular volume and mesangial cell counts per glomerulus compared to PBS. These effects were reversed with the addition of naloxone to morphine. (see Table) Morphine-stimulated cultured mouse mesangial cells underwent proliferation, which was inhibited with the addition of naloxone (202% ±14.3 vs. 108% ±12.9 increase over control, p<0.01). Morphine-stimulated mesangial cells had increased expression of phosphorylated STAT-3 by western blot analysis. Blockade of STAT-3 with PpYLKTK-mts (0.2mM) resulted in inhibition of morphine-stimulated mesangial cell proliferation (202% ±14.3 vs.110±29, p<0.01). In summary, morphine exposure markedly induced kidney growth in transgenic sickle mice, as evidenced by increased kidney size, glomerular volume, and glomerular cell counts. Furthermore, morphine stimulates a proliferative response in mesangial cells via STAT-3 signaling. These results suggest morphine may accelerate renal injury in sickle cell disease. Group Kidney Weight (g) Glomerular Volume (mm3) #MC/glomerulus Results (mean ± SD): λ p<0.01 vs. Sickle PBS; π p=0.01 vs. Sickle MS; γ p<0.01 vs. Sickle MS. Sickle PBS 0.190 ± 0.02 133 ± 7 27 ± 0.5 Sickle Naloxone 0.176 ± 0.01 124 ± 13 26 ± 1.2 Sickle Morphine 0.251 ± 0.04 λ 163 ± 14 λ 37 ± 1.9 λ Sickle MS+ Nal 0.193 ± 0.01 π 127 ± 12 π 30 ± 1.5 γ

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24

Green, David. "Thrombosis in sickle cell disease." Journal of Laboratory and Clinical Medicine 134, no. 4 (October 1999): 329–30. http://dx.doi.org/10.1016/s0022-2143(99)90145-2.

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25

Tomer, Aaron, Laurence A. Harker, Suha Kasey, and James R. Eckman. "Thrombogenesis in sickle cell disease." Journal of Laboratory and Clinical Medicine 137, no. 6 (June 2001): 398–407. http://dx.doi.org/10.1067/mlc.2001.115450.

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26

Brown, Douglas L., and Jeno I. Sebes. "Sickle cell gnathopathy: Radiologic assessment." Oral Surgery, Oral Medicine, Oral Pathology 61, no. 6 (June 1986): 653–56. http://dx.doi.org/10.1016/0030-4220(86)90114-3.

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27

Donadi, E. A., and R. P. Falcao. "Neutrophil chemotaxis in sickle cell anaemia, sickle cell beta zero thalassaemia, and after splenectomy." Journal of Clinical Pathology 40, no. 6 (June 1, 1987): 670–72. http://dx.doi.org/10.1136/jcp.40.6.670.

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28

Thogmartin, Jon R., Christopher I. Wilson, Noel A. Palma, Susan S. Ignacio, and William A. Pellan. "Histological Diagnosis of Sickle Cell Trait." American Journal of Forensic Medicine and Pathology 30, no. 1 (March 2009): 36–39. http://dx.doi.org/10.1097/paf.0b013e3181873835.

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29

Scheinin, Lisa, and Charles V. Wetli. "Sudden Death and Sickle Cell Trait." American Journal of Forensic Medicine and Pathology 30, no. 2 (June 2009): 204–8. http://dx.doi.org/10.1097/paf.0b013e318187dfcd.

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30

Omar, A. M., T. David, P. P. Pagare, M. S. Ghatge, Q. Chen, A. Mehta, Y. Zhang, et al. "Structural modification of azolylacryloyl derivatives yields a novel class of covalent modifiers of hemoglobin as potential antisickling agents." MedChemComm 10, no. 11 (2019): 1900–1906. http://dx.doi.org/10.1039/c9md00291j.

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The intracellular polymerization and the concomitant sickling processes, central to the pathology of sickle cell disease, can be mitigated by increasing the oxygen affinity of sickle hemoglobin (HbS).

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31

Chiadika, Simbo, Mary Lim-Fung, Fiorella Llanos-Chea, Astrid Serauto Canache, Wei Yang, Christina Paruthi, Xu Zhang, David D. McPherson, and Modupe Idowu. "Echocardiographic parameters to identify sickle cell patients with cardio-pathology." Echocardiography 35, no. 9 (May 14, 2018): 1271–76. http://dx.doi.org/10.1111/echo.14028.

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32

Hsu, Lewis, Bhalchandra Diwan, Jerrold M. Ward, and Constance T. Noguchi. "Pathology of “Berkeley” sickle-cell mice includes gallstones and priapism." Blood 107, no. 8 (April 15, 2006): 3414–15. http://dx.doi.org/10.1182/blood-2005-11-4500.

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Mascarenhas, Maria Inês, Helena Cristina Loureiro, Teresa Ferreira, and Alexandra Dias. "Sleep pathology characterization in sickle cell disease: Case-control study." Pediatric Pulmonology 50, no. 4 (July 10, 2014): 396–401. http://dx.doi.org/10.1002/ppul.23074.

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Mishra, Jayanti, Sanghamitra Pati, Mohammad Akhtar Hussain, Niraj Srivastava, and Sindhubala Mishra. "Screening for Sickle Cell Disease: A Hospital Based Study in Eastern Odisha, India." South Asian Journal of Experimental Biology 2, no. 2 (May 10, 2012): 57–60. http://dx.doi.org/10.38150/sajeb.2(2).p57-60.

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The highest frequency of sickle cell gene in India is reported in Odisha. The present study was taken up to assess the presence of sickle cell disease among febrile patients of a medical college of eastern Odisha. Patients referred from both pediatric and medicine department to the Hematology section of the department of Pathology, SCB Medical College, Cuttack were subjected to measurement of RBC indices, Sickling test, Haemoglobin Electrophoresis and Fetal Haemoglobin Estimation. Out of total 1000 referred patients 76(7.6%) were found to be positive for sickling. Twoâ€third of sicklingpositive patients had sickle cell trait with electrophoretic AS band. There was a significant association between age and positive sickling (χ2 = 24.357; df = 4, P = <0.0001). No significant association was observed between sickling and gender. Sickle cell positive cases are not uncommon in eastern Odisha. Our study demonstrated sickle cell trait to be more common among screened patients than other forms of sickle cell diseases.

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Townes, Tim M. "Gene Replacement Therapy for Sickle Cell Disease and Other Blood Disorders." Hematology 2008, no. 1 (January 1, 2008): 193–96. http://dx.doi.org/10.1182/asheducation-2008.1.193.

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Abstract Previous studies have demonstrated that sickle cell disease (SCD) can be corrected in mouse models by transduction of hematopoietic stem cells with lentiviral vectors containing anti-sickling globin genes followed by transplantation of these cells into syngeneic recipients. Although self-inactivating (SIN) lentiviral vectors with or without insulator elements should provide a safe and effective treatment in humans, some concerns about insertional mutagenesis persist. An ideal correction would involve replacement of the sickle globin gene (βS) with a normal copy of the gene (βA). We recently derived embryonic stem (ES) cells from a novel knockin mouse model of SCD and tested a protocol for correcting the sickle mutation by homologous recombination. Animals derived after gene replacement produced high levels of normal human hemoglobin (HbA), and the pathology associated with SCD was corrected. These experiments provided a foundation for similar studies in which our group collaborated with Rudolf Jaenisch’s laboratory to correct SCD by gene replacement in iPS (induced pluripotent stem) cells derived by direct reprogramming of sickle skin fibroblasts. Corrected iPS cells were differentiated into hematopoeitic progenitors that were transplanted into irradiated sickle recipients. The transplanted animals produced high levels of normal human HbA, and the pathology of SCD was corrected. These proof-of-principle studies provide a foundation for the development of gene replacement therapy for human patients with SCD and other blood disorders.

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Carden, Marcus A., Ross M. Fasano, and Emily Riehm Meier. "Not all red cells sickle the same: Contributions of the reticulocyte to disease pathology in sickle cell anemia." Blood Reviews 40 (March 2020): 100637. http://dx.doi.org/10.1016/j.blre.2019.100637.

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37

Keegan, Philip M., Sindhuja Surapaneni, and Manu O. Platt. "Sickle Cell Disease Activates Peripheral Blood Mononuclear Cells to Induce Cathepsins K and V Activity in Endothelial Cells." Anemia 2012 (2012): 1–7. http://dx.doi.org/10.1155/2012/201781.

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Sickle cell disease is a genetic disease that increases systemic inflammation as well as the risk of pediatric strokes, but links between sickle-induced inflammation and arterial remodeling are not clear. Cathepsins are powerful elastases and collagenases secreted by endothelial cells and monocyte-derived macrophages in atherosclerosis, but their involvement in sickle cell disease has not been studied. Here, we investigated how tumor necrosis alpha (TNFα) and circulating mononuclear cell adhesion to human aortic endothelial cells (ECs) increase active cathepsins K and V as a model of inflammation occurring in the arterial wall. ECs were stimulated with TNFα and cultured with peripheral blood mononuclear cells (PBMCs) from persons homozygous for sickle (SS) or normal (AA) hemoglobin. TNFα was necessary to induce cathepsin K activity, but either PBMC binding or TNFα increased cathepsin V activity. SS PBMCs were unique; they induced cathepsin K in ECs without exogenous TNFα (n=4,P<0.05). Inhibition of c-Jun N-terminal kinase (JNK) significantly reduced cathepsins K and V activation by 60% and 51%, respectively. Together, the inflammation and activated circulating mononuclear cells upregulate cathepsin activity through JNK signaling, identifying new pharmaceutical targets to block the accelerated pathology observed in arteries of children with sickle cell disease.

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Decastel, Monique, Yannick Leborgne-Samuel, Laure Alexandre, Guy Merault, and Camille Berchel. "Morphological features of the human umbilical vein in normal, sickle cell trait, and sickle cell disease pregnancies." Human Pathology 30, no. 1 (January 1999): 13–20. http://dx.doi.org/10.1016/s0046-8177(99)90294-0.

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39

Beckman, Joan D., John D. Belcher, Julie V. Vineyard, Chunsheng Chen, Julia Nguyen, M. Osita Nwaneri, M. Gerard O'Sullivan, Evin Gulbahce, Robert P. Hebbel, and Gregory M. Vercellotti. "Inhaled carbon monoxide reduces leukocytosis in a murine model of sickle cell disease." American Journal of Physiology-Heart and Circulatory Physiology 297, no. 4 (October 2009): H1243—H1253. http://dx.doi.org/10.1152/ajpheart.00327.2009.

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Carbon monoxide (CO) has anti-inflammatory properties. We previously reported that acute treatments with inhaled CO inhibit vascular inflammation and hypoxia-induced vasoocclusion in sickle cell disease mouse models. Therefore, we hypothesized that chronic CO inhalation would decrease vascular inflammation and organ pathology in a sickle cell disease mouse model. The treatment of sickle cell disease mice with 25 or 250 parts/million inhaled CO for 1 h/day, 3 days/wk for 8–10 wk significantly decreased the total mean white blood cell, neutrophil, and lymphocyte counts in peripheral blood. Eight weeks of 250 parts/million CO treatments reduced staining for myeloid and lymphoid markers in the bone marrow of sickle mice. Bone marrow from treated sickle mice exhibited a significant decrease in colony-forming unit granulocyte-macrophage during colony-forming cell assays. Anti-inflammatory signaling pathways phospho-Akt and phospho-p38 MAPK were markedly increased in CO-treated sickle livers. Importantly, CO-treated sickle mice had a significant reduction in liver parenchymal necrosis, reflecting the anti-inflammatory benefits of CO. We conclude that inhaled CO may be a beneficial anti-inflammatory therapy for sickle cell disease.

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Culberson, Donald E., Elizabeth A. Manci, Arvind K. Shah, Johnson Haynes, Samir K. Ballas, Charles Pegelow, and Elliot Vichinsky. "Nesidioblastosis in Sickle Cell Disease." Pediatric Pathology & Molecular Medicine 20, no. 2 (January 2001): 155–65. http://dx.doi.org/10.1080/15513810109168608.

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Inusa, Baba, Lewis Hsu, Neeraj Kohli, Anissa Patel, Kilali Ominu-Evbota, Kofi Anie, and Wale Atoyebi. "Sickle Cell Disease—Genetics, Pathophysiology, Clinical Presentation and Treatment." International Journal of Neonatal Screening 5, no. 2 (May 7, 2019): 20. http://dx.doi.org/10.3390/ijns5020020.

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Sickle cell disease (SCD) is a monogenetic disorder due to a single base-pair point mutation in the β-globin gene resulting in the substitution of the amino acid valine for glutamic acid in the β-globin chain. Phenotypic variation in the clinical presentation and disease outcome is a characteristic feature of the disorder. Understanding the pathogenesis and pathophysiology of the disorder is central to the choice of therapeutic development and intervention. In this special edition for newborn screening for haemoglobin disorders, it is pertinent to describe the genetic, pathologic and clinical presentation of sickle cell disease as a prelude to the justification for screening. Through a systematic review of the literature using search terms relating to SCD up till 2019, we identified relevant descriptive publications for inclusion. The scope of this review is mainly an overview of the clinical features of pain, the cardinal symptom in SCD, which present following the drop in foetal haemoglobin as young as five to six months after birth. The relative impact of haemolysis and small-vessel occlusive pathology remains controversial, a combination of features probably contribute to the different pathologies. We also provide an overview of emerging therapies in SCD.

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Thein, Mya S., Norris E. Igbineweka, and Swee Lay Thein. "Sickle cell disease in the older adult." Pathology 49, no. 1 (January 2017): 1–9. http://dx.doi.org/10.1016/j.pathol.2016.10.002.

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Graham, Jason K., Marina Mosunjac, Randy L. Hanzlick, and Mario Mosunjac. "Sickle Cell Lung Disease and Sudden Death." American Journal of Forensic Medicine and Pathology 28, no. 2 (June 2007): 168–72. http://dx.doi.org/10.1097/01.paf.0000257397.92466.50.

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Ansari, Junaid, and Felicity N. E. Gavins. "Ischemia-Reperfusion Injury in Sickle Cell Disease." American Journal of Pathology 189, no. 4 (April 2019): 706–18. http://dx.doi.org/10.1016/j.ajpath.2018.12.012.

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Embury, SH, MA Gholson, P. Gillette, and RF Rieder. "The leftward deletion alpha-thal-2 haplotype in a black subject with hemoglobin SS." Blood 65, no. 3 (March 1, 1985): 769–71. http://dx.doi.org/10.1182/blood.v65.3.769.769.

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Abstract We have identified a black individual with homozygous sickle cell anemia who is the silent carrier of alpha-thalassemia (genotype - alpha/alpha alpha) due to heterozygosity for the leftward deletion alpha-thal-2 haplotype. This deletion has not been described previously in a black subject and is the only leftward deletion that we have found among 255 alpha-thal-2 chromosomes from sickle cell subjects. Its effects on the clinical, hematologic, biosynthetic, and cellular pathology of sickle cell anemia resemble those reported for the common alpha-thalassemia genotypes of the black population.

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46

Embury, SH, MA Gholson, P. Gillette, and RF Rieder. "The leftward deletion alpha-thal-2 haplotype in a black subject with hemoglobin SS." Blood 65, no. 3 (March 1, 1985): 769–71. http://dx.doi.org/10.1182/blood.v65.3.769.bloodjournal653769.

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We have identified a black individual with homozygous sickle cell anemia who is the silent carrier of alpha-thalassemia (genotype - alpha/alpha alpha) due to heterozygosity for the leftward deletion alpha-thal-2 haplotype. This deletion has not been described previously in a black subject and is the only leftward deletion that we have found among 255 alpha-thal-2 chromosomes from sickle cell subjects. Its effects on the clinical, hematologic, biosynthetic, and cellular pathology of sickle cell anemia resemble those reported for the common alpha-thalassemia genotypes of the black population.

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Suragani, Rajasekhar NVS, Robert Li, Sharon M. Cawley, R. Scott Pearsall, and Ravindra Kumar. "Modified Actriib-Fc Fusion Protein (ACE-536) Mitigates Sickling and Red Cell Pathology in a Murine Model of Sickle Cell Disease." Blood 124, no. 21 (December 6, 2014): 4055. http://dx.doi.org/10.1182/blood.v124.21.4055.4055.

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Abstract Sickle cell disease (SCD) is a debilitating hereditary disorder caused by a single point mutation in the β-globin gene resulting in the production of sickle hemoglobin variant (HbS). In the deoxygenated state, HbS is labile and undergoes auto-oxidation and polymerizes to generate rigid and irreversibly sickled erythrocytes. The pathophysiology of SCD include increased red cell hemolysis, reactive oxygen species and phosphatidylserine (PS) exposure on RBC membranes which leads to a very short red cell half-life, increased reticulocytosis and splenomegaly. Sickled RBCs show enhanced adherence to activated endothelium causing chronic inflammation leading to frequent and acute painful vaso-occlusive crises in SCD patients. Hydroxyurea (HU) augments fetal hemoglobin production, decreases irreversible sickle cells and painful events, and is the only approved therapy for SCD patients. However, recent studies have shown dose limiting myelosupression with HU treatment. Approximately one-third of patients do not respond to HU therapy thereby highlighting the need for alternative treatment strategies. ACE-536 is a modified type IIB activin receptor-Fc fusion protein (ACE-536)1 which functions as a ligand trap for certain members of the TGFβ superfamily. In a murine model of β-thalassemia, RAP-536 (murine ortholog of ACE-536) treatment reduced hemichromes on RBC membranes, decreased reactive oxygen species, reduced hemolysis, improved red cell half-life and thus corrected anemia and mitigated disease complications of β-thalassemia syndrome2. In the present study, we evaluated RAP-536 as a monotherapy and combination therapy with HU in the murine model of sickle cell disease (βS/βS)3. SCD mice were dosed with RAP-536 (1 mg/kg, twice weekly, s.c.) or TBS vehicle (VEH) control (N=5/group) for 3 months. A combination treatment with HU (100mg/kg, i.p.) and RAP-536 (10mg/kg, s.c) twice weekly for 2 months was performed and compared with vehicle or HU monotherapy treated SCD mice. Non-symptomatic compound heterozygote (β/βS) littermates were treated similarly (N=5/group) and used as controls to confirm disease in SCD (βS/βS) mice. At study baseline, SCD mice had reduced RBC number (-28%, P<0.01) and hemoglobin (-14.5%, P<0.05) and increased reticulocytes (+50%, P<0.001) compared to compound heterozygote mice. Following one month of treatment, RAP-536 (1mg/kg) significantly reduced spleen weight (-20.5%, P<0.05), decreased serum bilirubin content (-17%, P<0.01) and cell free hemoglobin (-30.7%, P=0.06) compared to vehicle treated mice indicating decreased hemolysis. Most remarkably, blood smears from RAP-536 treated SCD mice displayed a decrease in number of irreversibly sickled erythrocytes (-66.5%, P<0.001) as well as reduced annexin V/PS exposure (-18.75%, N.S), suggesting improved membrane phospholipid asymmetry. RAP-536 treatment showed increased RBC number (+15.2%, P<0.01) and hemoglobin (+9.28%, P<0.05) compared to VEH treatment with concomitant decrease in reticulocytes (-13.5%, P< 0.05), suggestive of an increase in red cell half-life. Furthermore, histopathological analysis of spleen, kidneys and heart revealed a trend toward reduced intravascular congestion in RAP-536 treated SCD mice. Preliminary data from the combination treatment of HU and RAP-536 in SCD mice displayed additive beneficial effects as compared to HU alone. The combination of RAP-536 and HU produced a greater reduction in annexin V/PS exposure on peripheral blood cells than did HU alone compared to vehicle treatment (-35.6%, P<0.001 vs. -22.2%, N.S, respectively). Similarly, HU+RAP-536 showed a greater reduction in spleen size than HU alone (-50.7%, P<0.05 vs. -20.2%, N.S) respectively, compared to vehicle treated SCD mice. Additional analyses are in progress. Taken together, these data demonstrates that RAP-536 reduces the RBC sickling and red blood cell pathology in SCD and also shows its utility as monotherapy and in combination with HU to further mitigate the disease severity. ACE-536 is currently being tested in Phase 2 clinical trials in MDS and β-thalassemia patients, and merits evaluation as a therapy for SCD patients. References: Suragani RN et.al; Nature Medicine 2014; 20: 408-14Suragani RN et.al; Blood 2014; 123: 3864-72Wu LC etal; Blood. 2006; 108:1183-8. Disclosures Suragani: Acceleron Pharma Inc: Employment, Equity Ownership. Li:Acceleron Pharma Inc: Employment, Equity Ownership. Cawley:Acceleron Pharma Inc: Employment. Pearsall:Acceleron Pharma Inc: Employment, Equity Ownership. Kumar:Acceleron Pharma Inc: Employment, Equity Ownership.

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Culberson, Donald, Elizabeth Manci, Arvind Shah, Johnson Haynes, Samir Ballas, Charles Pegelow, and Elliot Vichinsky. "Nesidioblastosis in Sickle Cell Disease." Fetal and Pediatric Pathology 20, no. 2 (March 1, 2001): 155–65. http://dx.doi.org/10.3109/15513810109168608.

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49

Hammerschmidt, Dale E. "Sickle cell disease: What makes the red cells stay in the microvasculature long enough to sickle?" Journal of Laboratory and Clinical Medicine 144, no. 5 (November 2004): 227–28. http://dx.doi.org/10.1016/j.lab.2004.10.004.

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50

Keleku-Lukwete, Nadine, Mikiko Suzuki, Harit Panda, Akihito Otsuki, Fumiki Katsuoka, Ritsumi Saito, Daisuke Saigusa, Akira Uruno, and Masayuki Yamamoto. "Nrf2 activation in myeloid cells and endothelial cells differentially mitigates sickle cell disease pathology in mice." Blood Advances 3, no. 8 (April 23, 2019): 1285–97. http://dx.doi.org/10.1182/bloodadvances.2018017574.

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Abstract Sickle cell disease (SCD) is caused by a monogenic mutation of the β-globin gene and affects millions of people worldwide. SCD is associated with sustained hemolytic anemia, vasoocclusion, ischemia-reperfusion injury, oxidative tissue damage, inflammatory cell activation, and systemic endothelial dysfunction. The transcription factor Nrf2 coordinates the expression of a wide variety of genes encoding antioxidant, detoxification, and metabolic enzymes. Nrf2 participates in suppressing proinflammatory cytokines and organ protection in SCD. However, little is known regarding the mechanisms by which Nrf2 ameliorates SCD pathology or how some cells respond to Nrf2 stimuli to alleviate SCD pathology. Here, we asked whether monocytes/granulocytes and/or endothelial cells are particularly critical in alleviating the pathology of SCD. By targeting these cells with a Cre recombinase system, we generated SCD::Keap1F/F::LysM-Cre and Tie1-Cre mice with constitutive Nrf2 activation in monocytes/granulocytes and endothelial cells, respectively. Analyses of SCD::Keap1F/F::LysM-Cre and SCD::Keap1F/F::Tie1-Cre mice revealed significantly reduced inflammation, along with decreased white blood cell counts and lower Tnfα and Il1β expression in the lungs. Notably, SCD::Keap1F/F::LysM-Cre mice exhibited reduced heme distribution in the liver, consistent with a decrease in the damaged areas. Vascular function in SCD::Keap1F/F::Tie1-Cre mice was significantly improved, with a 50% decrease in vascular leakage and low expression of the adhesion molecules Vcam1 and P-selectin. Thus, Nrf2 activation in monocytes/granulocytes and endothelial cells contributes differentially and cooperatively to the improvement of SCD pathology.

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Journal articles on the topic 'Sickle cell pathology'

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