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Severe aplastic anemia

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https://www.readbyqxmd.com/read/29046127/serum-ferritin-is-a-different-predictor-from-transfusion-history-for-allogeneic-transplantation-outcome-in-patients-with-severe-aplastic-anemia
#1
Xiaoyu Zhang, Yuanyuan Shi, Yong Huang, Guixin Zhang, Yi He, Erlie Jiang, Jialin Wei, Donglin Yang, Qiaoling Ma, Rongli Zhang, Weihua Zhai, Sizhou Feng, Mingzhe Han
OBJECTIVES: Severe aplastic anemia (SAA) patients receive more red blood cell (RBC) transfusions as supportive management. We aim to clarify the associations between transfusion history or pre-transplantation serum ferritin (SF) and the overall survival of allogeneic hematopoietic stem cell transplantation (allo-HSCT) among SAA patients. MATERIAL AND METHODS: We retrospectively investigated 96 SAA patients undergoing allo-HSCT, and grouped them according to pre-transplantation duration...
October 18, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29037549/long-term-outcome-of-pregnancy-complicating-with-severe-aplastic-anemia-under-supportive-care
#2
Kuan-Ju Chen, Yao-Lung Chang, Horng Chang, Shen-Yuan Su, Hsiu-Huei Peng, Shuenn-Dyh Chang, An-Shine Chao
OBJECTIVES: Pregnancy associated with aplastic anemia (AA) is a rare and heterogeneous disorder. We aimed to identify and evaluate the maternal and pregnant outcomes of pregnancy-associated severe AA treated with supportive care. MATERIALS AND METHODS: A 25-year retrospective study was conducted at in a single center between 1990 and 2014 with pregnancy associated severe AA. In addition, relevant published cases of antenatally diagnosed pregnancy-associated severe AA after 1990 were identified by PubMed...
October 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29032265/effect-of-antithymocyte-globulin-source-on-outcomes-of-hla-matched-siblings-allogeneic-hematopoietic-stem-cell-transplantation-for-patients-with-severe-aplastic-anemia
#3
Xin Chen, Jialin Wei, Yong Huang, Yi He, Donglin Yang, Rongli Zhang, Erlie Jiang, Qiaoling Ma, Weihua Zhai, Jianfeng Yao, Guixin Zhang, Sizhou Feng, Mingzhe Han
OBJECTIVE: To evaluate efficacy of porcine antithymocyte globulin (ATG) in HLA-matched siblings allogeneic hematopoietic stem cell transplantation (MSD-HSCT) for patients with severe aplastic anemia (SAA). METHODS: The clinical data of 113 SAA patients who received MSD-HSCT from January 2005 to November 2016 were analyzed retrospectively. Of these, 58 patients received rabbit ATG as a part of conditioning regimen (R-ATG group), while the other 55 patients received porcine ATG (P-ATG group)...
October 12, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28988743/fludarabine-based-reduced-intensity-regimen-for-matched-related-donor-hematopoietic-stem-cell-transplantation-in-acquired-severe-aplastic-anemia
#4
K M Ghanem, M A Kharfan-Dabaja, H El-Solh, N Harris, N Kreidieh, R Nehme, S A Muwakkit, R Saab, A Bazarbachi, M R Abboud
Different conditioning regimens have been evaluated in matched-related donor allogeneic hematopoietic stem cell transplantation (allo-HSCT) for acquired severe aplastic anemia (SAA) with varying results. In this manuscript, we report our experience with fludarabine (120mg/m(2)), very low dose cyclophosphamide (1200mg/m(2)) and antithymocyte globulin (7.5mg/kg). Low dose total body irradiation (2Gy) was added to the conditioning regimen for patients older than 15 years. Nineteen patients (median age 23years) underwent transplant between 2008 and 2015...
October 4, 2017: Current Research in Translational Medicine
https://www.readbyqxmd.com/read/28971166/somatic-hla-mutations-expose-the-role-of-class-i-mediated-autoimmunity-in-aplastic-anemia-and-its-clonal-complications
#5
Daria V Babushok, Jamie L Duke, Hongbo M Xie, Natasha Stanley, Jamie Atienza, Nieves Perdigones, Peter Nicholas, Deborah Ferriola, Yimei Li, Hugh Huang, Wenda Ye, Jennifer J D Morrissette, Jane Kearns, David L Porter, Gregory M Podsakoff, Laurence C Eisenlohr, Jaclyn A Biegel, Stella T Chou, Dimitrios S Monos, Monica Bessler, Timothy S Olson
Acquired aplastic anemia (aAA) is an acquired deficiency of early hematopoietic cells, characterized by inadequate blood production, and a predisposition to myelodysplastic syndrome (MDS) and leukemia. Although its exact pathogenesis is unknown, aAA is thought to be driven by Human Leukocyte Antigen (HLA)-restricted T cell immunity, with earlier studies favoring HLA class II-mediated pathways. Using whole exome sequencing (WES), we recently identified two aAA patients with somatic mutations in HLA class I genes...
October 10, 2017: Blood Advances
https://www.readbyqxmd.com/read/28968996/apc-c-is-essential-for-hematopoiesis-and-impaired-in-aplastic-anemia
#6
Jia Wang, Min-Zhi Yin, Ke-Wen Zhao, Fang Ke, Wen-Jie Jin, Xiao-Lin Guo, Tian-Hui Liu, Xiao-Ye Liu, Hao Gu, Xiao-Min Yu, Zhen Li, Li-Li Mu, Deng-Li Hong, Jing Chen, Guo-Qiang Chen
Anaphase promoting complex/cyclosome (APC/C) is essential for cell cycle progression. Recently, its non-mitotic functions were also reported but less studied in several tissues including hematopoietic cells. Here, we developed an inducible Anapc2 (a core subunit of APC/C) knockout mice. The animals displayed a fatal bone marrow failure within 7 days after knockout induction. Their hematopoietic stem and progenitor cells (HSPCs) demonstrated a sharp decline and could form little colony. Further, the results of BrdU label-retaining cell assay showed that the dormant HPSCs lost rapidly...
September 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28954343/-improved-outcome-by-optimized-conditioning-regimens-with-an-increased-dose-of-cyclophosphamide-in-allogeneic-peripheral-blood-stem-cell-transplantation-for-severe-aplastic-anemia
#7
Y Huang, Y He, X Liu, D L Yang, R L Zhang, E L Jiang, Q L Ma, J L Wei, S Z Feng, M Z Han
Objective: To respectively analyze the impact of conditioning regimens with a dose-decreased cyclophosphamide (Cy) on the outcome in fully matched sibling donor (MSD) peripheral blood stem cell transplantation (PBSCT) for severe aplastic anemia (SAA) . Methods: Two conditioning regimens with different doses of Cy (150 mg/kg or 120 mg/kg) in combination with fludarabine (Flu) and antithymocyte globulin (ATG) for MSD-PBSCT were investigated in 51 patients with acquired SAA. Results: Overall survival and failure-free survival in patients received 150 mg/kg of Cy (Cy150 cohort) or 120 mg/kg (Cy120 cohort) were 93...
August 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28951259/chimerism-in-pediatric-hematopoietic-stem-cell-transplantation-and-its-correlation-with-the-clinical-outcome
#8
Hala Gabr, Ilham Youssry, Yasmin El-Ansary, Ghada Mosallam, Nermine Magdi Riad, Mariam Onsy F Hanna
Hematopoietic stem cell transplantation (HSCT) is the only hope to cure many inherited and acquired hematological disorders in children. Monitoring of chimerism helps to predict the post-transplantation events, with the intention to enhance the long-term disease free survival (DFS). The study aimed to investigate the importance of early chimerism detection to predict the clinical outcome following HSCT. The study included nine recipients (six β-thalassemia and three severe aplastic anemia patients) and their 10/10 HLA identical sibling donors...
September 23, 2017: Transplant Immunology
https://www.readbyqxmd.com/read/28941711/persistent-elevation-of-plasma-thrombopoietin-levels-after-treatment-in-severe-aplastic-anemia
#9
Xin Zhao, Xingmin Feng, Zhijie Wu, Thomas Winkler, Ronan Desmond, Matthew Olnes, Bogdan Dumitriu, Danielle M Townsley, Cynthia E Dunbar, Neal S Young
Although hematopoietic growth factors are found at high levels in aplastic anemia (AA) patients, little is known about their dynamic change over time after treatment. We examined plasma concentrations of hematopoietic growth factors sequentially in 55 severe AA patients, including 45 treatment-naive patients who had received immunosuppressive therapy (IST) or IST and eltrombopag and 10 IST-refractory patients who had received eltrombopag only, focusing on thrombopoietin (TPO). TPO concentrations were much higher than normal in patients before treatment and then decreased in responders but not in nonresponders...
September 20, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28900860/modified-immunosuppressive-therapy-with-porcine-antilymphocyte-globulin-plus-delayed-cyclosporine-a-in-children-with-severe-aplastic-anemia
#10
Qingya Cui, Pingping Sha, Haifei Chen, Hongshi Shen, Longmei Qin, Zhengyang Li, Tianqin Wu, Zhaoyue Wang
Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine (CsA) is the standard treatment for children with severe aplastic anemia (SAA) with no human leukocyte antigen-matched siblings. Due to the unavailability of horse ATG in China, porcine antilymphocyte globulin (p-ALG), which is less expensive and more effective than rabbit ATG, is widely used. We sought to evaluate the efficacy and safety profile of modified IST with p-ALG plus delayed CsA at day 21 in 50 SAA children. Eighteen SAA patients who progressed from nonsevere aplastic anemia (NSAA) were classified as SAA-II; the other 32 patients were classified as SAA-I...
September 12, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28877042/hematopoietic-cell-transplantation-for-aplastic-anemia
#11
Rajat Kumar, Carmem Bonfim, Biju George
PURPOSE OF REVIEW: Improvements in allogeneic hematopoietic cell transplantation (HCT) with better donor selection, conditioning regimens and graft vs. host disease prophylaxis make it reasonable to move HCT earlier in the algorithm for management of severe aplastic anemia (SAA). Recent progress in transplantation is reviewed whereas issues related to developing countries are also addressed. RECENT FINDINGS: Multiple research centers are reporting on clonality, mutations and telomere disorders in SAA, which may help to choose the most appropriate therapy upfront...
November 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28860001/immune-mediated-hematopoietic-failure-after-allogeneic-hematopoietic-stem-cell-transplantation-a-common-cause-of-late-graft-failure-in-patients-with-complete-donor-chimerism
#12
Kana Maruyama, Nobuyuki Aotsuka, Yoshihisa Kumano, Naoko Sato, Naomi Kawashima, Yoshiyuki Onda, Hiroyuki Maruyama, Takamasa Katagiri, Yoshitaka Zaimoku, Noriharu Nakagawa, Kazuyoshi Hosomichi, Seishi Ogawa, Shinji Nakao
Late graft failure (LGF) without evidence of residual recipient cells is a serious complication after allogeneic hematopoietic stem cell transplantation (allo-SCT) and often requires stem cell infusion from the same donor when the patient fails to respond to conventional therapies. We screened the peripheral blood (PB) of 14 patients who developed donor-type LGF at 2 to 132 months after allo-SCT for the presence of the markers for immune-mediated bone marrow (BM) failure. Increased glycosylphosphatidyl inositol-anchored protein-deficient (GPI-AP(-)) leukocytes, which accounted for ...
August 30, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28815305/predictors-of-early-mortality-after-rabbit-antithymocyte-globulin-as-first-line-treatment-in-severe-aplastic-anemia
#13
Elias H Atta, Carlos B L Lima, Danielle S P Dias, Diego V Clé, Mariana M Bonduel, Gabriela B Sciuccati, Larissa A Medeiros, Michel M Oliveira, Marco A Salvino, Marlene P Garanito, Patricia B Blum Fonseca, Sara Teresinha O Saad, Rodrigo T Calado, Phillip Scheinberg
Despite being recommended as first-line immunosuppressive therapy in severe aplastic anemia (SAA), horse antithymocyte globulin (ATG) is still unavailable in many countries outside the USA. Rabbit ATG is more lymphocytoxic than horse ATG, and this might result in a higher incidence of severe infections and early mortality. This study was designed to identify the risk factors for early mortality and overall survival (OS) after rabbit ATG in patients with SAA. We retrospectively reviewed 185 patients with SAA who underwent rabbit ATG and cyclosporine...
August 16, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28813500/telomere-biology-and-telomerase-mutations-in-cirrhotic-patients-with-hepatocellular-carcinoma
#14
Flávia S Donaires, Natália F Scatena, Raquel M Alves-Paiva, Joshua D Podlevsky, Dhenugen Logeswaran, Barbara A Santana, Andreza C Teixeira, Julian J-L Chen, Rodrigo T Calado, Ana L C Martinelli
Telomeres are repetitive DNA sequences at linear chromosome termini, protecting chromosomes against end-to-end fusion and damage, providing chromosomal stability. Telomeres shorten with mitotic cellular division, but are maintained in cells with high proliferative capacity by telomerase. Loss-of-function mutations in telomere-maintenance genes are genetic risk factors for cirrhosis development in humans and murine models. Telomerase deficiency provokes accelerated telomere shortening and dysfunction, facilitating genomic instability and oncogenesis...
2017: PloS One
https://www.readbyqxmd.com/read/28805792/immune-reconstitution-in-patients-with-acquired-severe-aplastic-anemia-after-haploidentical-stem-cell-transplantation
#15
X-Y Pei, X-Y Zhao, L-P Xu, Y Wang, X-H Zhang, Y-J Chang, X-J Huang
Immune recovery (IR) after haploidentical stem cell transplantation (haplo-SCT) in severe aplastic anemia (SAA) patients remains relatively unknown. In this study, we examined immune cell subset counts and immunoglobulins in 81 SAA patients from day 30 to day 365 after haplo-SCT. Simultaneously, we determined which factors influence IR and analyzed the effects of immune cell subsets on transplant outcomes. We found that: (i) The reconstitution of different immune cell subsets occurred at different rates after haplo-SCT...
August 14, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28802588/t-cell-costimulation-blockade-promotes-transplantation-tolerance-in-combination-with-sirolimus-and-post-transplantation-cyclophosphamide-for-haploidentical-transplantation-in-children-with-severe-aplastic-anemia
#16
Sarita Rani Jaiswal, Prakash Bhakuni, Shamsuz Zaman, Satish Bansal, Priyanka Bharadwaj, Sneh Bhargava, Suparno Chakrabarti
We conducted a pilot study employing extended T cell costimulation blockade (COSBL) with Abatacept along with sirolimus and post-transplantation cyclophosphamide (PTCy) in 10 patients (median age 12) with severe aplastic anemia (SAA). Nine patients engrafted in the COSBL group, compared to all 10 patients (median 14 vs 13days) treated on PTCy protocols without abatacept (CONTROL group). The incidence of acute graft-versus-host disease (GVHD) was 10.5% in the COSBL group compared to 50% in the CONTROL group (p=0...
August 9, 2017: Transplant Immunology
https://www.readbyqxmd.com/read/28797779/long-term-outcomes-of-cord-blood-transplantation-from-an-hla-identical-sibling-for-patients-with-bone-marrow-failure-syndromes-a-report-from-eurocord-cord-blood-committee-and-severe-aplastic-anemia-working-party-of-the-european-society-for-blood-and-marrow
#17
Simona Pagliuca, Régis Peffault de Latour, Fernanda Volt, Franco Locatelli, Marco Zecca, Jean-Hugues Dalle, Patrizia Comoli, Kim Vettenranta, Miguel Angel Diaz, Or Reuven, Yves Bertrand, Cristina Diaz de Heredia, Arnon Nagler, Ardeshir Ghavamzadeh, Sabina Sufliarska, Sarah Lawson, Chantal Kenzey, Vanderson Rocha, Carlo Dufour, Eliane Gluckman, Jakob Passweg, Annalisa Ruggeri
Cord blood transplantation (CBT) from HLA-identical siblings is an attractive option for patients with bone marrow failure (BMF) syndrome because of the low risk of graft-versus-host disease (GVHD) and the absence of risk to the donor. We analyzed outcomes of 117 patients with inherited or acquired BMF syndrome who received CBT from a related HLA-identical donor in European Society for Blood and Marrow Transplantation centers between 1988 and 2014. Ninety-seven patients had inherited and 20 patients acquired BMF syndrome...
August 7, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28751565/incidence-and-outcome-of-acquired-aplastic-anemia-real-world-data-from-patients-diagnosed-in-sweden-from-2000-2011
#18
Krista Vaht, Magnus Göransson, Kristina Carlson, Cecilia Isaksson, Stig Lenhoff, Anna Sandstedt, Bertil Uggla, Jacek Winiarski, Per Ljungman, Mats Brune, Per-Ola Andersson
A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70-80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000-2011...
October 2017: Haematologica
https://www.readbyqxmd.com/read/28728262/-evaluation-of-efficacy-of-immunosuppressive-therapy-plus-recombinant-human-thrombopoietin-for-children-with-severe-aplastic-anemia
#19
K Zhou, C X Liu, Y Li, J P Li, H H Fan, L Zhang, L P Jing, G X Peng, L Ye, Y Li, L Song, X Zhao, W R Yang, Z J Wu, F Chen, F K Zhang
Objective: To evaluate the therapeutic efficacy and safety of immunosuppressive therapy (IST) combined with recombinant human thrombopoietin (rhTPO) for severe aplastic anemia (SAA) in pediatric patients. Method: A retrospective case-control study was conducted and the clinical data of 45 pediatric patients with de novo SAA admitted to the Anemia Diagnosis and Treatment Center of Chinese Academy of Medical Sciences & Blood Disease Hospital during the period from December 2009 to December 2014 were analyzed...
July 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28715625/-myelofibrosis-in-a-benzene-exposed-cleaning-worker
#20
Roser Bausà, Lydia Navarro, Imma Cortès-Franch
Long-term exposure to benzene has been associated with several blood malignancies, including aplastic anemia, myeloproliferative neoplasms, and different leukemias. We present a case of primary myelofibrosis in a 59-year-old woman who worked as a cleaner at a car dealership and automobile mechanic shop. For 25 years, she used gasoline as a degreaser and solvent to clean engine parts, floors and work desks on a daily basis. She was referred by her primary care provider to the Occupational Health Unit of Barcelona to assess whether her illness was work-related...
July 2017: Archivos de Prevención de Riesgos Laborales
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