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Severe aplastic anemia

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https://www.readbyqxmd.com/read/28341733/effect-of-antithymocyte-globulin-source-on-outcomes-of-bone-marrow-transplantation-for-severe-aplastic-anemia
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
https://www.readbyqxmd.com/read/28128288/the-spleen-microenvironment-influences-disease-transformation-in-a-mouse-model-of-kit-d816v-dependent-myeloproliferative-neoplasm
#10
Natalie Pelusi, Maike Kosanke, Tamara Riedt, Corinna Rösseler, Kristin Seré, Jin Li, Ines Gütgemann, Martin Zenke, Viktor Janzen, Hubert Schorle
Activating mutations leading to ligand-independent signaling of the stem cell factor receptor KIT are associated with several hematopoietic malignancies. One of the most common alterations is the D816V mutation. In this study, we characterized mice, which conditionally express the humanized KIT(D816V) receptor in the adult hematopoietic system to determine the pathological consequences of unrestrained KIT signaling during blood cell development. We found that KIT(D816V) mutant animals acquired a myeloproliferative neoplasm similar to polycythemia vera, marked by a massive increase in red blood cells and severe splenomegaly caused by excessive extramedullary erythropoiesis...
January 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28121342/clinical-applications-of-haploidentical-hematopoietic-stem-cell-transplantation-in-severe-aplastic-anemia
#11
P Zhang, K Feng, Y Xue, C-X Zhang, Y Wang, X-L Li
OBJECTIVE: The purpose of this study was to investigate the efficacy and safety of haploidentical allogeneic hemopoietic stem cell transplantation (allo-HSCT) in severe aplastic anemia (SAA) and prophylaxis of complications involved. PATIENTS AND METHODS: 8 patients with clinically diagnosed SAA (5 cases of SAA-I and 3 cases of SAA-II) were recruited, with the parents as the donors of hemopoietic stem cells. The conditioning regimen before HSCT included cyclophosphamide, fludarabine, pig anti-human lymphocyte immune globulin (p-ALG) and/or total body irradiation (TBI)...
January 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28115201/life-and-death-of-yeast-telomerase-rna
#12
REVIEW
Yulia Vasianovich, Raymund J Wellinger
Telomerase reverse transcriptase elongates telomeres to overcome their natural attrition and allow unlimited cellular proliferation, a characteristic shared by stem cells as well as the majority of malignant cancerous cells. The telomerase holoenzyme comprises a core RNA molecule, a catalytic protein subunit and other accessory proteins. Malfunction of certain telomerase components can cause serious genetic disorders including dyskeratosis congenita and aplastic anemia. A hierarchy of tightly regulated steps constitute the process of telomerase biogenesis, which if interrupted or misregulated, can impede production of a functional enzyme and severely affect telomere maintenance...
January 20, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/28107815/upfront-haploidentical-transplant-for-acquired-severe-aplastic-anemia-registry-based-comparison-with-matched-related-transplant
#13
Lan-Ping Xu, Song Jin, Shun-Qing Wang, Ling-Hui Xia, Hai Bai, Su-Jun Gao, Qi-Fa Liu, Jian-Min Wang, Xin Wang, Ming Jiang, Xi Zhang, De-Pei Wu, Xiao-Jun Huang
BACKGROUND: Haploidentical donor (HID) hematopoietic stem cell transplantation (HSCT) is an alternative treatment method for severe aplastic anemia (SAA) patients lacking suitable identical donors and those who are refractory to immunosuppressive therapy (IST). The current study evaluated the feasibility of upfront haploidentical HSCT in SAA patients. METHODS: We conducted a multicenter study based on a registry database. One hundred fifty-eight SAA patients who underwent upfront transplantation between June 2012 and September 2015 were enrolled...
January 21, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28100318/-clinical-characteristics-of-clonal-evolution-after-immunosuppressive-therapy-in-children-with-severe-very-severe-aplastic-anemia
#14
Jing-Liao Zhang, Tian-Feng Liu, Li-Xian Chang, Xia Chen, Yuan-Yuan Ren, Cong-Cong Sun, Chao Liu, Wen-Bin An, Yang Wan, Xiao-Juan Chen, Wen-Yu Yang, Shu-Chun Wang, Ye Guo, Yao Zou, Yu-Mei Chen, Xiao-Fan Zhu
OBJECTIVE: To evaluate the clinical characteristics and risk factors of clonal evolution after immunosuppressive therapy (IST) in children with severe/very severe aplastic anemia (SAA/VSAA). METHODS: The clinical data of 231 children with newly-diagnosed SAA/VSAA who received IST were retrospectively studied. The incidence and risk factors of clonal evolution after IST were analyzed. RESULTS: The 5-year overall survival rate of the 231 patients was 82...
January 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28097850/-a-regenerative-anemia-in-infants-2-cases-of-pearson%C3%A2-s-syndrome
#15
José M Martínez de Zabarte Fernández, Carmen Rodríguez-Vigil Iturrate, Cristina Martínez Faci, Inmaculada García Jiménez, Laura Murillo Sanjuan, Ascensión Muñoz Mellado
Anemia is very common in infants. Although its causes are usually not severe and treatable, proper etiologic diagnosis should be established. When anemia is non-regenerative, it can be caused by aplastic anemia, myelodysplastic syndrome, bone marrow infiltration or hematopoietic factors deficiencies. Another possible cause is Pearson's syndrome, a rare mitochondrial disease that causes non-regenerative anemia associated with other cytopenias, pancreatic insufficiency, lactic acidosis and great variability in clinical presentation conditioned by heteroplasmy...
February 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28096088/how-i-treat-acquired-aplastic-anemia
#16
Andrea Bacigalupo
Acquired severe aplastic anemia (SAA) is a rare hematologic disease associated with significant morbidity and mortality. Immune destruction of hemopoietic stem cells, plays an important role in the pathogenesis, as shown by successful treatment with immunosuppressive agents (IST), leading to transfusion independence, or complete recovery of peripheral blood counts, in a proportion of patients. Growth factors, can be combined with IST, and may improve response rates, as recently shown with thrombopoietin analogs...
January 17, 2017: Blood
https://www.readbyqxmd.com/read/28090056/suspected-pulmonary-infection-with-trichoderma-longibrachiatum-after-allogeneic-stem-cell-transplantation
#17
Tomoaki Akagi, Chizuko Kawamura, Norio Terasawa, Kohei Yamaguchi, Kohmei Kubo
Aspergillus and Candida species are the main causative agents of invasive fungal infections in immunocompromised human hosts. However, saprophytic fungi are now increasingly being recognized as serious pathogens. Trichoderma longibrachiatum has recently been described as an emerging pathogen in immunocompromised patients. We herein report a case of isolated suspected invasive pulmonary infection with T. longibrachiatum in a 29-year-old man with severe aplastic anemia who underwent allogeneic stem cell transplantation...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28088971/-eltrombopag-for-refractory-thrombocytopenia-in-patients-with-allogeneic-hematopoietic-stem-cell-transplantation
#18
Y R Ma, X J Huang, X D Mo, W Han, C H Yan, Y Chen, Y Ji, Y Y Chen, Y Wang, X H Zhang, K Y Liu, L P Xu
Objective: To evaluate the efficacy and safety of eltrombopag in post-HSCT thrombocytopenia. Methods: A total of 10 patients who underwent post-HSCT thrombocytopenia at Peking University center, who had been treated with eltrombopag, were retrospectively evaluated. Results: Of the 10 cases, 5 males and 5 females with a median of 34 years old (range, 17-54 years), 5 patients were acute myeloid leukemia, 3 with acute lymphoid leukemia and 2 with severe aplastic anemia. Nine patients had undergone haplo-identical donor transplantation, and one patient was a matched related recipient...
December 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28088966/-delayed-hematologic-response-to-immunosuppressive-therapy-in-severe-aplastic-anemia
#19
Y Yang, W R Yang, Z J Wu, X Zhao, L Zhang, L P Jing, K Zhou, Y Li, G X Peng, Y Li, J P Li, L Song, L Ye, H H Fan, F K Zhang
Objective: To explore the characteristics of delayed hematologic response in very/severe aplastic anemia (V/SAA) patients who were treated with immunosuppressive treatment (IST) as first-line approach, and investigate the rationality of early salvage treatment in refractory patients. Methods: The data of V/SAA patients front-line treated with IST were retrospectively analyzed. Delayed response was defined as acquiring hematologic response between 6 and 12 months after 1 course of IST. The clinical as well as hematologic characteristics of the delayed responded patients were investigated...
December 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28078653/meta-analysis-of-treatment-with-rabbit-and-horse-antithymocyte-globulin-for-aplastic-anemia
#20
Jin Hayakawa, Junya Kanda, Yu Akahoshi, Naonori Harada, Kazuaki Kameda, Tomotaka Ugai, Hidenori Wada, Yuko Ishihara, Koji Kawamura, Kana Sakamoto, Masahiro Ashizawa, Miki Sato, Kiriko Terasako-Saito, Shun-Ichi Kimura, Misato Kikuchi, Rie Yamazaki, Shinichi Kako, Yoshinobu Kanda
Aplastic anemia patients who received rabbit antithymocyte globulin exhibited response and survival rates inferior to those who received horse antithymocyte globulin in several studies. Therefore, we conducted a meta-analysis to compare rabbit and horse antithymocyte globulin as immunosuppressive therapy for aplastic anemia. We searched online databases for studies that compared antithymocyte globulin regimens as first-line treatment for aplastic anemia, including both randomized and non-randomized controlled trials...
January 11, 2017: International Journal of Hematology
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