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Severe aplastic anemia

Prabhsimranjot Singh, Ankur Sinha, Anisha Kamath, Sonali Malhotra
No abstract text is available yet for this article.
October 18, 2016: American Journal of Therapeutics
Tomomi Kogiso, Etsuko Hashimoto, Taito Ito, Toshifumi Hara, Yuichi Ikarashi, Kazuhisa Kodama, Makiko Taniai, Nobuyuki Torii, Kentaro Yoshinaga, Satoru Morita, Yutaka Takahashi, Junji Tanaka, Shuji Sakai, Masakazu Yamamoto, Katsutoshi Tokushige
A 56-year-old man was diagnosed with aplastic anemia and paroxysmal nocturnal hemoglobinuria at 43 years of age and treatment with cyclosporin A was started. Liver cirrhosis, ascites, and thrombus in the hepatic veins were found at 56 years of age and Budd-Chiari syndrome (BCS) was diagnosed according to angiography findings. He was treated with diuretics and paracentesis was performed several times, but with limited efficacy. A Denver(®) peritoneovenous shunt (PVS) was inserted into the right jugular vein; his ascites and renal function improved immediately and his general condition has remained good for 12 months since starting the above treatment regimen...
2016: Internal Medicine
Kensuke Usuki
Treatments of aplastic anemia are comprised of supportive therapy and aplastic anemia-specific therapy aimed at restoring hematopoiesis. Supportive therapies include transfusion, G-CSF, and the administration of iron chelation agents, as well as dealing specifically with individual symptoms. Aplastic anemia-specific treatments given with the aim of achieving hematopoietic recovery include immunosuppressive therapy, allogeneic hematopoietic stem cell transplantation, and anabolic hormone therapy. Although transplantation provides complete recovery of hematopoiesis (cure), there is a risk of death due to transplant-related complications...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
X X Li, M Wang, Y Q Shao, N Nie, J Shi, Z D Huang, J Zhang, M L Ge, J B Huang, Y Z Zheng
Objective: To assess the clinical feature and outcomes of severe aplastic anemia (SAA) patients suffered from bacteremia following antithymocyte globulin (ATG). Methods: A total of 264 cases hospitalized in our hospital between Jan 2000 and July 2011 were enrolled into this study. We evaluated the associated pathogens of bacteremia, analyzed the risk factors by Logistic regression and estimated the overall survival (OS) by Kaplan-Meier method for the cohort of patients. Results: Bloodstream infections occurred in 49 patients, with a median age of 20 (4-62) years, including 38 cases with very SAA (VSAA) and 11 SAA patients...
September 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Alberto Mussetti, Nancy A Kernan, Susan E Prockop, Andromachi Scaradavou, Rachel Lehrman, Julianne M Ruggiero, Kevin Curran, Rachel Kobos, Richard O'Reilly, Farid Boulad
Nonmalignant hematologic disorders (NMHD) of childhood comprise a variety of disorders, including acquired severe aplastic anemia and inherited marrow failure syndromes. Patients with high-risk NMHD without matched related donors fare poorly with allogeneic hematopoietic alternative donor stem cell transplantation (allo-HSCT) and are at high risk for developing graft-versus-host disease following unmodified grafts. The authors retrospectively analyzed data on 18 patients affected by NMHD, lacking a human leukocyte antigen (HLA)-identical sibling donor, who underwent an alternative donor allo-HSCT at their institution between April 2005 and May 2013...
October 7, 2016: Pediatric Hematology and Oncology
Zhi Guo, Hong-Yan Gao, Tian-Yan Zhang, Xiao-Dong Liu, Kai Yang, Jing-Xing Lou, Xue-Peng He, Yuan Zhang, Peng Chen, Hui-Ren Chen
The study was aimed to explore the efficacy and safety of allo-HSCT with high-dose cyclophosphamide-induced immune tolerance for SAA. In the present study, 20 cases (12 male, 8 female; average age = 17.8 years) received reduced-intensity conditioning allo-HSCT from August 2012 to August 2014 in the Beijing Military Region General Hospital. All were HLA mismatched and received CSA; 11 received ATG-intensive immune therapy. Donors underwent mobilization with cell colony-stimulating factor. The modified preconditioning regimen included reduced-strength fludarabine combined with Busulfex and cytarabine, cyclophosphamide...
October 5, 2016: International Journal of Hematology
Mehwesh Taj, Tayyaba Shah, Syeda Kanwal Aslam, Sidra Zaheer, Faryal Nawab, Sumaira Shaheen, Kashif Shafique, Tahir Sultan Shamsi
AIM: Aplastic anemia (AA) affects the Asian population two to three fold more than people in other regions. Besides the host genetics and socioeconomic status, several other environmental exposures have been linked with an AA etiology. We aimed to examine the association of various environmental exposures with AA occurrence among Pakistani individuals. SUBJECTS AND METHODS: A case-control study was conducted in Karachi, Pakistan, where cases (diagnosed AA patients) were selected from the National Institute of Blood Disease and Bone Marrow Transplantation (NIBD), while for each case, a single control (who was free of AA and visited the outpatient department of the same hospital for the treatment of minor ailments) was selected matched by age and sex...
2016: Zeitschrift Für Gesundheitswissenschaften, Journal of Public Health
Su Han Lum, John D Grainger
Aplastic anemia (AA) is a potential life-threatening hematopoietic stem cell (HSC) disorder resulting in cytopenia. The mainstays of treatment for AA are definitive therapy to restore HSCs and supportive measures to ameliorate cytopenia-related complications. The standard definitive therapy is HSC transplantation for young and medically fit patients with suitable donors and immunosuppressive therapy (IST) with antithymocyte globulin and cyclosporine for the remaining patients. A significant proportion of patients are refractory to IST or relapse after IST...
2016: Drug Design, Development and Therapy
Y B Choi, E S Yi, J W Lee, K W Sung, H H Koo, K H Yoo
We compared the outcomes of immunosuppressive treatment (IST) with those of alternative donor hematopoietic stem cell transplantation (HSCT) in children and adolescents with severe aplastic anemia (SAA). The medical records of 42 patients with SAA who received frontline IST (N=19) or frontline HSCT with an alternative donor (N=23) between 1998 and 2012 were analyzed retrospectively. Six patients responded in the frontline IST group, whereas 11 underwent salvage HSCT after IST failure. Twenty-one of 23 patients who underwent frontline HSCT survived without treatment failure...
September 26, 2016: Bone Marrow Transplantation
Shahinaz M Gadalla, Tao Wang, Casey Dagnall, Michael Haagenson, Stephen R Spellman, Belynda Hicks, Kristine Jones, Hormuzd A Katki, Stephanie J Lee, Sharon A Savage
We previously showed an association between donor leukocyte relative telomere length (RTL) and post-hematopoietic cell transplantation (HCT) survival in patients with severe aplastic anemia (SAA) who received bone marrow grafts at ages <40 years. Here, we tested the generalizability of the prior findings in an independent validation cohort and by recipient age and stem cell source in the combined discovery and validation cohorts. We used monoplex quantitative real-time PCR to measure RTL in: (1) a new SAA validation cohort of 428 patients (age range, ...
September 15, 2016: Biology of Blood and Marrow Transplantation
George E Georges, Rainer Storb
PURPOSE OF REVIEW: There has been a steady improvement in outcomes with allogeneic bone marrow transplantation (BMT) for severe aplastic anemia (SAA), because of progress in optimization of the conditioning regimens, donor hematopoietic cell source, and supportive care. Here, we review recently published data that highlight the improvements and current issues in the treatment of SAA. RECENT FINDINGS: Approximately one-third of aplastic anemia patients treated with immune suppressive therapy (IST) have acquired mutations in myeloid cancer candidate genes...
November 2016: Current Opinion in Hematology
S Wirths, W Bethge, J C Henes
According to experimental animal models and experiences of patients with coexisting autoimmune diseases, allogeneic stem cell transplantation has the potential to reestablish and maintain immunological tolerance. On the other hand, it is associated with significant treatment related mortality and may induce diverse immunological diseases by graft-versus-host reaction. Other than with severe aplastic anemia, it is not an established therapy for autoimmune diseases; it is under investigation in clinical trials and might be considered in severe, refractory immune cytopenia...
October 2016: Zeitschrift Für Rheumatologie
J Zhou, Yanli Zhang, Y W Fu, X H Zhu, L J Liang, Y L Zu, Q Wang, F K Yu, L J Han, B J Fang, X D Wei, Y P Song
OBJECTIVE: To evaluate the efficacy of allogeneic hematopoietic stem cell transplantation (allo- HSCT) for patients with severe aplastic anemia (SAA). METHODS: A retrospective study was conducted in 41 SAA patients received allo-HSCT from Oct. 2001 to May 2015. There were 27 males and 14 females with median age of 17(2-43) years old. Of them, 24 received matched sibling donor HSCT, 17 received unrelated donor transplantation. RESULTS: Hematopoiesis reconstitution was achieved in 38 patients (92...
August 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Hiroaki Shimizu, Nobuhiko Kobayashi, Masahiro Mihara, Hirono Iriuchishima, Takuma Ishizaki, Yoshihisa Kojima, Hiroshi Handa
Epstein-Barr virus-associated lymphoproliferative disorder (EBV-LPD) is a currently emerging serious complication in immunosuppressed patients, especially in allogeneic transplant recipients. Several fatal cases of EBV-LPD have been reported in aplastic anemia (AA) patients receiving immunosuppressive therapy (IST) with antithymocyte globulin (ATG) plus cyclosporine A (CsA), but no appropriate prophylactic or therapeutic strategy has been established. Herein, we describe a 29-year-old man whose EBV-LPD was successfully treated with rituximab...
2016: Acta Haematologica
Rumesh Chandar, Venkatesh Chandrasekaran, Barath Jagadisan, Delhi Kumar, Niranjan Biswal
A 7-year-old male child with very severe aplastic anemia developed refractory disease, which was attributed to febrile hemolytic transfusion reactions and was treated with immunosuppressants, which lead to a transient improvement. However, the child worsened subsequently and succumbed to an underlying hemophagocytic lymphohistiocytosis that was recognized late due to an overlap of clinical and biochemical parameters in both the conditions. Hemophagocytic lymphohistiocytosis should be an early suspicion in children with refractory very severe aplastic anemia and the detection of underlying gene mutations can predict disease severity...
August 19, 2016: Journal of Pediatric Hematology/oncology
J P Li, W R Yang, Y Li, L Ye, K Zhou, L P Jing, Y Li, G X Peng, L Song, F K Zhang, L Zhang
OBJECTIVE: To evaluate the efficacy and safety of intensive immunosupressive therapy (IST) with antithymocyte/antilymphocyte globulin plus cyclosporine A in the treatment of older patients (≥60 years) with severe aplastic anemia (SAA). METHODS: The hematologic response and safety of sixteen older SAA patients treated with IST regimen in our hospital were retrospectively analyzed , and the factors affecting response were also explored. RESULTS: A total of 16 older SAA patients were involved, the median age was 63...
July 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Amélie Fradet-Turcotte, Justine Sitz, Damien Grapton, Alexandre Orthwein
Maintaining genomic integrity is essential to preserve normal cellular physiology and to prevent the emergence of several human pathologies including cancer. The breast cancer susceptibility gene 2 (BRCA2, also known as the Fanconi anemia (FA) complementation group D1 (FANCD1)) is a potent tumor suppressor that has been extensively studied in DNA double-stranded break (DSB) repair by homologous recombination (HR). However, BRCA2 participates in numerous other processes central to maintaining genome stability, including DNA replication, telomere homeostasis and cell cycle progression...
October 2016: Endocrine-related Cancer
Linya You, Lin Li, Jinfeng Zou, Kezhi Yan, Jad Belle, Anastasia Nijnik, Edwin Wang, Xiang-Jiao Yang
Hematopoietic stem cells (HSCs) serve as a life-long reservoir for all blood cell types and are clinically useful for a variety of HSC transplantation-based therapies. Understanding the role of chromatin organization and regulation in HSC homeostasis may provide important insights into HSC development. Bromodomain- and PHD finger-containing protein 1 (BRPF1) is a multivalent chromatin regulator that possesses 4 nucleosome-binding domains and activates 3 lysine acetyltransferases (KAT6A, KAT6B, and KAT7), suggesting that this protein has the potential to stimulate crosstalk between different chromatin modifications...
September 1, 2016: Journal of Clinical Investigation
Weiwei Qi, Li Yan, Chunyan Liu, Rong Fu, Huaquan Wang, Zonghong Shao
Severe aplastic anemia (SAA) is a rare autoimmune disease characterized by severe pancytopenia and bone marrow failure, which is caused by activated T lymphocytes. In the present study, we evaluated histone H3 acetylation levels of bone marrow CD8(+) T cells in SAA patients, and analyzed its correlation with clinical condition parameters. We found that the percentages of CD8(+) T cell histone H3 acetylation in patients with untreated SAA, recovering SAA (R-SAA) and normal control, were 1.21 ± 0.08, 1.05 ± 0...
August 2, 2016: International Journal of Hematology
S Gallo, A E Woolfrey, L M Burroughs, B E Storer, M E D Flowers, P Hari, M A Pulsipher, S Heimfeld, H-P Kiem, B M Sandmaier, R Storb
A total of 21 patients with severe aplastic anemia (SAA) underwent marrow transplantation from HLA-identical siblings following a standard conditioning regimen with cyclophosphamide (50 mg/kg/day × 4 days) and horse antithymocyte globulin (30 mg/kg/day × 3 days). Post-grafting immunosuppression consisted of a short course of methotrexate (MTX) combined with cyclosporine (CSP). The transplant protocol tested the hypothesis that the incidence of chronic GvHD could be reduced by limiting the marrow grafts to ⩽2...
August 1, 2016: Bone Marrow Transplantation
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