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Severe aplastic anemia

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https://www.readbyqxmd.com/read/28522577/shorter-leukocyte-telomere-length-is-associated-with-higher-risk-of-infections-a-prospective-study-of-75-309-individuals-from-the-general-population
#1
Jens Helby, Børge G Nordestgaard, Thomas Benfield, Stig E Bojesen
In the general population, older age is associated with short leukocyte telomere length and with high risk of infections. In a recent study of allogeneic hematopoietic cell transplantation for severe aplastic anemia, long donor leukocyte telomere length was associated with improved survival in the recipients. These findings suggest that leukocyte telomere length could possibly be a marker of immune competence. Therefore, we tested the hypothesis that shorter leukocyte telomere length is associated with higher risk of infectious disease hospitalization and infection related death...
May 18, 2017: Haematologica
https://www.readbyqxmd.com/read/28512588/severe-aplastic-anemia-following-parvovirus-b19-associated-acute-hepatitis
#2
Masanori Furukawa, Kosuke Kaji, Hiroyuki Masuda, Kuniaki Ozaki, Shohei Asada, Aritoshi Koizumi, Takuya Kubo, Norihisa Nishimura, Yasuhiko Sawada, Kosuke Takeda, Tsuyoshi Mashitani, Masayuki Kubo, Itsuto Amano, Tomoyuki Ootani, Chiho Ohbayashi, Koji Murata, Tatsuichi Ann, Akira Mitoro, Hitoshi Yoshiji
Human parvovirus (HPV) B19 is linked to a variety of clinical manifestations, such as erythema infectiosum, nonimmune hydrops fetalis, and transient aplastic anemia. Although a few cases have shown HPVB19 infection as a possible causative agent for hepatitis-associated aplastic anemia (HAAA) in immunocompetent patients, most reported cases of HAAA following transient hepatitis did not have delayed remission. Here we report a rare case of severe aplastic anemia following acute hepatitis with prolonged jaundice due to HPVB19 infection in a previously healthy young male...
2017: Case Reports in Hepatology
https://www.readbyqxmd.com/read/28481355/population-pharmacokinetics-of-fludarabine-in-patients-with-aplastic-anemia-and-fanconi-anemia-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#3
E Mohanan, J C Panetta, K M Lakshmi, E S Edison, A Korula, N A Fouzia, A Abraham, A Viswabandya, V Mathews, B George, A Srivastava, P Balasubramanian
Although hematopoietic stem cell transplantation (HSCT) with a conditioning regimen consisting of fludarabine (F-araA) and cyclophosphamide (Cy) is associated with improved outcome in young patients with aplastic anemia (AA) and Fanconi anemia (FA), several factors limit the success of the procedure. We evaluated the population pharmacokinetics (POPPK) of F-araA and its influence on HSCT outcome in patients (n=53) with AA and FA undergoing HSCT. Patients carrying a 5'-UTR polymorphism in NT5E gene (rs2295890 G>C) exhibited significantly lower plasma F-araA clearance compared to those with wild-type genotype (7...
May 8, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28473904/from-chronic-immune-thrombocytopenia-to-severe-aplastic-anemia-recent-insights-into-the-evolution-of-eltrombopag
#4
REVIEW
Harinder Gill, Raymond S M Wong, Yok-Lam Kwong
Thrombopoietin (TPO) is the most potent cytokine stimulating thrombopoiesis. Therapy with exogenous TPO is limited by the formation of antibodies cross-reacting with endogenous TPO. Mimetics of TPO are compounds with no antigenic similarity to TPO. Eltrombopag is an orally-active nonpeptide small molecule that binds to the transmembrane portion of the TPO receptor MPL. Initial trials of eltrombopag have centered on immune thrombocytopenia (ITP), which is due to both increased destruction and decreased production of platelets...
May 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28468095/-the-mechanism-of-bone-marrow-derived-mesenchymal-stem-cells-excessive-senescence-in-severe-aplastic-anemia-mouse-model
#5
Y Q Ou, H Y Liu, W Lu, M J Wen, H Liu
Objective: To explore the mechanism of excessive senescence in bone marrow-derived mesenchymal stem cells (BM-MSC) of mouse model with severe aplastic anemia (SAA) . Methods: 40 BALB/c mice were randomly assigned to two groups of control (n=20) and AA (n=20) . SAA mouse model was induced by intraperitoneal injection with IFN-γ and intragastric infusion with busulfan. BM-MSC were isolated and cultured from bone marrow of SAA and healthy mice. The cell morphology was observed by inverted microscope and cell cytoskeleton was stained by Rhodamine-Phalloidin; The level of proliferation was analyzed by CCK-8 method, and cell cycle was tested by flow cytometry...
April 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28443261/diabetes-mellitus-caused-by-secondary-hemochromatosis-after-multiple-blood-transfusions-in-2-patients-with-severe-aplastic-anemia
#6
Hyun Jin Kim, Yoon-Myung Kim, Eungu Kang, Beom Hee Lee, Jin-Ho Choi, Han-Wook Yoo
Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT) at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9...
March 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28436973/a-review-of-hematopoietic-cell-transplantation-in-china-data-and-trends-during-2008-2016
#7
L-P Xu, D-P Wu, M-Z Han, H Huang, Q-F Liu, D-H Liu, Z-M Sun, L-H Xia, J Chen, H-X Wang, C Wang, C-F Li, Y-R Lai, J-M Wang, D-B Zhou, H Chen, Y-P Song, T Liu, K-Y Liu, X-J Huang
Hematopoietic cell transplantation (HCT) activity in China was surveyed to assess its current status. A record number of HCTs (21 884: 16 631 allogeneic (76%) and 5253 autologous (24%)) were reported by 76 centers in China between 1 January 2008 and 30 June 2016. HCT trends included continued growth in transplant activity, a continued rapid increase in haploidentical donors (HID), and slower growth for unrelated donors, matched-related donors (MRD) and cord blood transplantation (CBT). The proportion of HID HCT among allogeneic HCTs increased from 29...
April 24, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28423296/eltrombopag-added-to-standard-immunosuppression-for-aplastic-anemia
#8
Danielle M Townsley, Phillip Scheinberg, Thomas Winkler, Ronan Desmond, Bogdan Dumitriu, Olga Rios, Barbara Weinstein, Janet Valdez, Jennifer Lotter, Xingmin Feng, Marie Desierto, Harshraj Leuva, Margaret Bevans, Colin Wu, Andre Larochelle, Katherine R Calvo, Cynthia E Dunbar, Neal S Young
BACKGROUND: Acquired aplastic anemia results from immune-mediated destruction of bone marrow. Immunosuppressive therapies are effective, but reduced numbers of residual stem cells may limit their efficacy. In patients with aplastic anemia that was refractory to immunosuppression, eltrombopag, a synthetic thrombopoietin-receptor agonist, led to clinically significant increases in blood counts in almost half the patients. We combined standard immunosuppressive therapy with eltrombopag in previously untreated patients with severe aplastic anemia...
April 20, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28385068/transforming-growth-factor-15-increased-in-severe-aplastic-anemia-patients
#9
Yuanyuan Shao, Honglei Wang, Chunyan Liu, Qiuying Cao, Rong Fu, Huaquan Wang, Ting Wang, Weiwei Qi, Zonghong Shao
OBJECTIVES: The patients with severe aplastic anemia (SAA) usually rely on red cell transfusion which lead to secondary iron overload. Transforming growth differentiation factor-15 (GDF-15) plays an important role in erythropoiesis and iron regulation. In this study, we investigated the level of GDF-15 and other indexes of iron metabolism in SAA patients to explore the correlation with GDF-15 and iron overload in SAA. METHODS: The levels of serum GDF-15, hepcidin (Hepc), and erythropoietin (EPO) were determined by ELISA...
April 6, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28376627/severe-acute-hepatitis-and-cold-agglutinin-related-hemolytic-anemia-secondary-to-prime-infection-with-epstein-barr-virus
#10
Guillermo Ontanilla Clavijo, Julia Praena Segovia, Álvaro Giráldez Gallego, María Elisa Cordero Matía, José Manuel Sousa Martín
Epstein-Barr virus, a member of the Herpesviridae family, is responsible for the infectious mononucleosis clinical syndrome, which mainly includes the pharyngitis, fever, and lymphadenopathy triad after incubation for 30-50 days. The liver is involved in 80-90% of patients in a self-limiting transient manner, with jaundice being much more uncommon (5%). From a hematological standpoint it may manifest aplastic anemia, neutropenia, and thrombocytopenia. We report a case of infectious mononucleosis that included severe acute hepatitis and was associated with severe hemolytic anemia secondary to cold agglutinins...
April 3, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28361276/aplastic-anemia-in-a-lung-adenocarcinoma-patient-receiving-pemetrexed
#11
Goushi Matama, Takaaki Tokito, Hiroaki Takeoka, Yuki Hiraoka, Norikazu Matsuo, Masayuki Nakamura, Hidenobu Ishii, Takashi Kinoshita, Koichi Azuma, Kazuhiko Yamada, Tomoaki Hoshino
Pemetrexed (PEM) is an antimetabolite drug that interferes with enzymes involved in DNA synthesis and also the folate-dependent metabolic processes necessary for DNA replication and homocysteine homeostasis. Continuation maintenance with PEM after induction therapy with PEM plus cisplatin has been the standard form of first-line chemotherapy for advanced non-squamous non-small cell lung cancer. The regimen has a low incidence of bone marrow suppression, and the incidences of anemia, leukopenia, neutropenia and thrombocytopenia exceeding grade 3 are less than 5%...
March 30, 2017: Investigational New Drugs
https://www.readbyqxmd.com/read/28341733/effect-of-antithymocyte-globulin-source-on-outcomes-of-bone-marrow-transplantation-for-severe-aplastic-anemia
#12
Natasha Kekre, Ying Zhang, Mei-Jie Zhang, Jeanette Carreras, Parvez Ahmed, Paolo Anderlini, Elias Hallack Atta, Mouhab Ayas, Jaap Jan Boelens, Carmem Bonfim, H Joachim Deeg, Neena Kapoor, Jong-Wook Lee, Ryotaro Nakamura, Michael A Pulsipher, Mary Eapen, Joseph H Antin
For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin results in more profound immunosuppression. We analyzed 833 severe aplastic anemia transplants between 2008 and 2013 using HLA-matched siblings (n=546) or unrelated donors (n=287) who received antithymocyte globulin as part of their conditioning regimen and bone marrow graft...
March 24, 2017: Haematologica
https://www.readbyqxmd.com/read/28279029/-how-i-standardly-diagnose-and-treat-severe-aplastic-anemia
#13
Z H Shao
No abstract text is available yet for this article.
February 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28270348/myelodysplasia-and-acute-myeloid-leukemia-fifteen-years-after-high-dose-cyclophosphamide-in-a-child-with-severe-aplastic-anemia
#14
José Carlos Jaime-Pérez, Liliana Nataly Guerra-Leal, Olga Graciela Cantú-Rodríguez, David Gómez-Almaguer
No abstract text is available yet for this article.
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28245775/allogeneic-hematopoietic-stem-cell-transplant-for-severe-aplastic-anemia-current-state-and-future-directions
#15
Qixin Sun, Bingyi Wu, Zhigang Zhu, Can Sun, Jingxia Xu, Hui Long, Yuxian Huang, Jianhui Xu, Chaoyang Song
Acquired severe aplastic anemia (SAA) is a rare and life-threatening bone marrow failure syndrome characterized by cytotoxic T-cells excessive activity, hematopoietic precursors decrease and peripheral blood (PB) pancytopenia. Patients with severe aplastic anemia (SAA) died 1 to 2 years after diagnosis due to fatal infections and/or hemorrhagic complications if they went without any effective treatment. Nowadays, Immunosuppressive therapy (IST) and allogeneic hematopoietic stem cell transplantation (HSCT) are still the standard treatment for SAA...
February 27, 2017: Current Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28221210/cutaneous-emboli-of-invasive-basidiomycosis-in-a-child-with-aplastic-anemia
#16
Desmond Shi-Wei Lim, Poh-Lin Tan, Roland Jureen, Kong-Bing Tan
Invasive fungal diseases are a significant cause of mortality among the immunocompromised. This report documents an unusual case of disseminated fungal infection in a child with severe aplastic anemia. The offending fungus, a Basidiomycete, is rarely known to cause human infections. The patient presented acutely with multiple purpuric skin lesions in various parts of the body. The skin biopsy revealed septated fungal hyphae embolized within small dermal blood vessels. Molecular sequencing indicated Earliella scabrosa as the likely organism...
March 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28194076/outcome-of-cyclosporine-monotherapy-in-patients-of-aplastic-anemia-experience-of-a-tertiary-care-hospital-in-eastern-india
#17
Prakas Kumar Mandal, Suvraneel Baul, Tuphan Kanti Dolai, Rajib De, Prantar Chakrabarti
INTRODUCTION: Immune suppression is a crucial pillar for treatment of aplastic anemia. Cyclosporine monotherapy is an easily available, affordable therapeutic option with good safety profile. METHODS AND MATERIALS: This prospective study was conducted over a period of 2 years from June 2012 to July 2014. The diagnosis and response to treatment of aplastic anemia was established as per published criteria. Follow up was done at 3 and 6 months in order to assess the response...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28178130/de-escalation-empirical-antibiotic-therapy-improved-survival-for-patients-with-severe-aplastic-anemia-treated-with-antithymocyte-globulin
#18
COMPARATIVE STUDY
Rong Fu, Tong Chen, Jia Song, Guojin Wang, Lijuan Li, Erbao Ruan, Hui Liu, Yihao Wang, Huaquan Wang, Limin Xing, Yuhong Wu, Hong Liu, Wen Qu, Zonghong Shao
We aimed to investigate the efficacy and safety of de-escalation empirical therapy for controlling infection in patients with severe aplastic anaemia (SAA) treated with antithymocyte globulin (ATG). Eighty-seven ATG-treated SAA patients who had microbiological culture-confirmed infections from 2006 to 2015 in our center were retrospectively analyzed. The efficacy of de-escalation and non-de-escalation therapy was compared. Among all 87 patients, 63 patients were treated with de-escalation therapy and 24 patients with non-de-escalation therapy...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28164595/differential-expression-of-cd52-cd14-and-hla-dr-on-cd4-monocytes-in-three-types-of-acquired-bone-marrow-failure-syndromes
#19
Yang Kai, Dai Yuanyuan, Guan Shihe, Mu Xuanxuan, Zhang Hao, Pan Ying, Wu Yuanyuan, Wang Aihua, Sun Beibei, TongYang, Zhou Tingdong
BACKGROUND: Aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH), and myelodysplastic syndrome (MDS) are the common spectrums of acquired bone marrow failure syndromes (BMFs). Accurate and timely diagnosis is a significant clinical challenge because of the overlapping features. The pathogenesis is not fully understood, but several studies have suggested that defective monocyte functions play an important role. We aimed to find whether the different expressions of CD52, CD14 and HLA-DR on CD4+ monocytes would be helpful in the preliminary diagnosis of acquired BMFs...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28154281/fulminant-laryngeal-tracheobronchial-pulmonary-aspergillosis-a-rare-and-fatal-complication-in-allogeneic-hematopoietic-stem-cell-transplantation-recipients
#20
Tao Tao, Ying-Hui Zhang, Sheng-Li Xue, De-Pei Wu, Feng Chen
A 23-year-old man who had previously undergone allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe aplastic anemia was diagnosed with invasive laryngeal-tracheobronchial-pulmonary aspergillosis after presenting with a persistent dry cough at six months post-transplantation based on the findings of laryngoscopy and fiberoptic bronchoscopy. A fiberoptic bronchoscope was used to remove the obstructive material from the patient's airway and posaconazole plus caspofungin were administered to successfully to treat the patient...
2017: Internal Medicine
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