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https://www.readbyqxmd.com/read/28107566/recent-advances-in-understanding-clonal-haematopoiesis-in-aplastic-anaemia
#1
REVIEW
Natasha Stanley, Timothy S Olson, Daria V Babushok
Acquired aplastic anaemia (AA) is an immune-mediated bone marrow failure disorder inextricably linked to clonal haematopoiesis. The majority of AA patients have somatic mutations and/or structural chromosomal abnormalities detected as early as at diagnosis. In contrast to other conditions linked to clonal haematopoiesis, the clonal signature of AA reflects its immune pathophysiology. The most common alterations are clonal expansions of cells lacking glycophosphotidylinositol-anchored proteins, loss of human leucocyte antigen alleles, and mutations in BCOR/BCORL1, ASXL1 and DNMT3A...
January 20, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28100318/-clinical-characteristics-of-clonal-evolution-after-immunosuppressive-therapy-in-children-with-severe-very-severe-aplastic-anemia
#2
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/28098254/nanoscale-measurement-of-trace-element-distributions-in-spartina-alterniflora-root-tissue-during-dormancy
#3
Huan Feng, Yu Qian, J Kirk Cochran, Qingzhi Zhu, Wen Hu, Hanfei Yan, Li Li, Xiaojing Huang, Yong S Chu, Houjun Liu, Shinjae Yoo, Chang-Jun Liu
This paper reports a nanometer-scale investigation of trace element (As, Ca, Cr, Cu, Fe, Mn, Ni, S and Zn) distributions in the root system Spartina alterniflora during dormancy. The sample was collected on a salt marsh island in Jamaica Bay, New York, in April 2015 and the root was cross-sectioned with 10 μm resolution. Synchrotron X-ray nanofluorescence was applied to map the trace element distributions in selected areas of the root epidermis and endodermis. The sampling resolution was 60 nm to increase the measurement accuracy and reduce the uncertainty...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28097850/-a-regenerative-anemia-in-infants-2-cases-of-pearson%C3%A2-s-syndrome
#4
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...
1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28096088/how-i-treat-acquired-aplastic-anemia
#5
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
#6
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
#7
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
#8
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/28081344/peripheral-flt-3-ligand-levels-as-a-pathobiological-parameter-duringthe-clinical-course-of-acute-myeloid-leukemia
#9
Memduh Şahin, İbrahim Celalettin Haznedaroğlu, Demircan Özbalcı
BACKGROUND/AIM: FLT-3 ligand is a growth factor affecting the hematopoietic lineage. The aim of this study was to evaluate the variability of peripheral FLT-3 ligand during the clinical course of acute myeloid leukemia (AML) patients. MATERIALS AND METHODS: Twenty-four patients were enrolled in this study in order to assess alterations in the circulating levels of FLT-3 ligand during the clinical course of AML. RESULTS: We studied the association in the diagnostic period between the FLT-3 ligand and peripheral blood cells together with serum electrolytes...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28078653/meta-analysis-of-treatment-with-rabbit-and-horse-antithymocyte-globulin-for-aplastic-anemia
#10
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
https://www.readbyqxmd.com/read/28075434/diagnosis-and-surgical-correction-of-congenital-bilateral-patellar-luxation-in-two-dwarf-zebu-calves
#11
Göknil Kalayci, Cagri Binici, Julia Tünsmeyer, Silke Stein, Ahmad Al Aiyan, Kerstin Müller, Leo Brunnberg
This case study presents two female dwarf zebu calves with permanent bilateral flexion of the stifle joint secondary to bilateral congenital lateral patellar luxation. The patella could be manually repositioned bilaterally in the first calf (14 days old), but re-luxated immediately with release of manual pressure (luxation grade III). In the second calf (28 days old) both patellae were permanently luxated laterally (grade IV). The right and left stifles of each calf were operated in a staged procedure with approximately 14 days between surgery of the two sides...
January 11, 2017: Tierärztliche Praxis. Ausgabe G, Grosstiere/Nutztiere
https://www.readbyqxmd.com/read/28067873/cotransplantation-of-bone-marrow-derived-mesenchymal-stem-cells-in-haploidentical-hematopoietic-stem-cell-transplantation-in-patients-with-severe-aplastic-anemia-an-interim-summary-for-a-multicenter-phase-ii-trial-results
#12
Z Liu, Y Zhang, H Xiao, Z Yao, H Zhang, Q Liu, B Wu, D Nie, Y Li, Y Pang, Z Fan, L Li, Z Jiang, F Duan, H Li, P Zhang, Y Gao, L Ouyang, C Yue, M Xie, C Shi, Y Xiao, S Wang
Haploidentical hematopoietic stem cell transplantation (haplo-HSCT) for severe aplastic anemia (SAA) is mainly limited by the high incidence of graft failure and GvHD. Mesenchymal stem cells (MSCs) have been shown to support hematopoiesis in vivo and to display potent immunosuppressive effects to prevent or treat GvHD after HSCT. In a multicenter phase II trial, we developed an approach with co-transplantation of MSCs in patients undergoing haplo-HSCT. Forty-four patients with SAA were included. The conditioning regimen included busulfan, cyclophosphamide and thymoglobulin (ATG)...
January 9, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28062362/induced-pluripotent-stem-cell-technology-a-window-for-studying-the-pathogenesis-of-acquired-aplastic-anemia-and-possible-applications
#13
REVIEW
Mahmoud Elbadry, J Luis Espinoza, Shinji Nakao
Recent progress in human induced pluripotent stem cells (iPSCs) has opened the door to better understand the biology of human diseases, especially in rare disorders, such as acquired aplastic anemia (AA), in which the target hematopoietic tissues are depleted. The advent of somatic cell reprogramming has presented new routes for generating hematopoietic stem cells (HSCs) from patient-derived iPSCs and their differentiation into hematopoietic lineages. The purpose of this review is to discuss the recent advances in iPSC research technology and its potential applications in disease modeling for understanding the pathogenesis of bone marrow failure syndrome (BMFS) and the potential clinical utility of iPSC-derived cells...
January 3, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28060175/cochlear-nerve-deficiency-and-brain-abnormalities-in-pediatric-patients
#14
Thierry Morlet, Markian Pazuniak, Robert C O'Reilly, Vinay Kandula, Arabinda K Choudhary
HYPOTHESIS: To investigate the intracranial abnormalities present in children with cochlear nerve deficiency (CND), including abnormalities of other cranial nerves, and to describe their auditory abilities. BACKGROUND: The prevalence of CND has increased with the development of high resolution magnetic resonance imaging (MRI). There are varying degrees of CND from true aplasia to hypoplasia. The etiology of CND remains unclear and it may be associated with intracranial abnormalities in some instances...
January 4, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28060106/interleukin-6-and-interleukin-8-levels-correlate-with-the-severity-of-aplastic-anemia-in-children
#15
Vineeta Gupta, Sushil Kumar, Rimjhim Sonowal, Surya K Singh
AIM: The aim of this study was to evaluate the levels of interleukin (IL)-6 and IL-8 in patients with aplastic anemia and its correlation with severity of the disease. MATERIALS AND METHODS: IL-6 and IL-8 levels were measured in 40 patients with aplastic anemia in the age group of 4 to 14 years. A total of 40 healthy children served as controls. Quantitative estimation of IL-6 and IL-8 was performed using a solid-phase sandwich ELISA kit. Results were presented as IL-6 and IL-8 concentrations in pg/mL...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28040785/circulating-micrornas-promising-biomarkers-in-aplastic-anemia
#16
EDITORIAL
Jonathan B Bell, Sameem Abedin, Leonidas C Platanias
No abstract text is available yet for this article.
January 2017: Haematologica
https://www.readbyqxmd.com/read/28035550/bleeding-after-endoscopic-procedures-in-patients-with-chronic-hematologic-thrombocytopenia
#17
Hyun Jin Oh, Jae Myung Park, Seung Bae Yoon, Han Hee Lee, Chul-Hyun Lim, Jin Su Kim, Yu Kyung Cho, Bo-In Lee, Young-Seok Cho, Myung-Gyu Choi
BACKGROUND: Procedure-induced bleeding is a major complication after endoscopic intervention. AIMS: The aim of this study was to investigate the risk of endoscopy-related bleeding in patients with chronic hematologic thrombocytopenia. METHODS: We investigated endoscopy-related bleeding in 175 procedures performed on 108 patients with immune thrombocytopenic purpura or aplastic anemia. The outcomes were compared with those of 350 procedures on age-, sex-, and procedure-matched control subjects...
December 29, 2016: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28030425/features-of-hepatitis-in-hepatitis-associated-aplastic-anemia-clinical-and-histopathologic-study
#18
Kalyani R Patel, Alison Bertuch, Ghadir S Sasa, Ryan W Himes, Hao Wu
OBJECTIVES: Hepatitis-associated aplastic anemia (HAA) is a rare variant of aplastic anemia in which patients present with severe pancytopenia after an episode of acute hepatitis. The marrow failure is often rapid, severe, and usually fatal if untreated. The preceding hepatitis is largely under-studied. METHODS: Retrospective study of the clinical and histopathologic features of hepatitis in pediatric patients who subsequently developed aplastic anemia and comparison with consecutive cases of acute liver failure and random cases of autoimmune hepatitis during the same time frame...
January 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28025876/novel-deletion-mutation-of-hla-b-40-02-gene-in-acquired-aplastic-anemia
#19
T-D Jeong, Y-C Mun, H-S Chung, D Seo, J Im, J Huh
Despite prevalence of clonal evolution in patients with aplastic anemia (AA), somatic mutation of human leukocyte antigen (HLA) gene is rarely reported. Herein, we reported a case of acquired AA (aAA) harboring a new four-base-pair deletion mutation within exon 4 of HLA-B*40:02 leading to frameshift and premature stop codon. The HLA-B*40:02 mutant allele was detected in the patient's peripheral blood sample not in patient's buccal epithelial cells. The patient received allogenic hematopoietic stem cell transplantation (HSCT) from HLA-matched sibling donor...
January 2017: HLA
https://www.readbyqxmd.com/read/28024501/-effect-of-rabbit-anti-human-thymocyte-immunoglobulin-combined-with-cyclosporin-a-on-severe-aplastic-anemia
#20
Wei Luo, Lin-Hua Ji, Hui Geng, Xiao-Jing Ma, Hua Xiong, Qi-Chao Yin, Jie Ma
OBJECTIVE: To study the efficacy and safety of anti-human thymocyte immunoglobulin(ATG-F) combined with cyclosporin A(CsA) on patients with severe aplastic anemia (SSA), so as to provide support for clinical work. METHODS: From January 2010 to December 2015, 78 patients with SAA admitted in our hospital were divided into 2 groups: ATG-F+CsA group(40 cases) and ATG-F group(38 cases). After treatment for 6 months, the effective rate, side reaction rate and time of effect initiation were compared between 2 groups...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
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