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Myeloid aplastic

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https://www.readbyqxmd.com/read/28212262/molecular-cytogenetic-approach-to-characterize-novel-and-cryptic-chromosome-abnormalities-in-childhood-myeloid-malignances-of-fanconi-anemia
#1
Maria L R Borges, Roberto R Capela de Matos, Bethânia D A Silva Amaral, Eliane M Soares-Ventura, Edinalva P Leite, Mariluze O D Silva, Maria T M Nogueira Cornélio, Maria L M Silva, Thomas Liehr, Terezinha D J Marques-Salles
Myeloid malignancies can be either primary or secondary, whether or not a specific cause can be determined. Fanconi anemia (FA), a rare constitutional bone marrow failure, usually presents an increased possibility of clonal evolution, due to the increase in chromosomal instability, TP53 activation, and cell death. The evolution of FA may include aplastic anemia by the progressive failure of the bone marrow and myelod neoplasias, such as acute myeloid leukemia and myelodysplastic syndrome. Chromosome abnormalities, particularly of chromosomes, 1, 3, and 7, during the aplastic phase of the disease are predictive of evolution to acute myeloid leukemia/myelodysplastic syndrome...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28129526/addition-of-androgens-improves-survival-in-elderly-patients-with-acute-myeloid-leukemia-a-goelams-study
#2
Arnaud Pigneux, Marie C Béné, Philippe Guardiola, Christian Recher, Jean-Francois Hamel, Mathieu Sauvezie, Jean-Luc Harousseau, Olivier Tournilhac, Francis Witz, Christian Berthou, Martine Escoffre-Barbe, Denis Guyotat, Nathalie Fegueux, Chantal Himberlin, Mathilde Hunault, Martine Delain, Bruno Lioure, Eric Jourdan, Frederic Bauduer, Francois Dreyfus, Jean-Yves Cahn, Jean-Jacques Sotto, Norbert Ifrah
Purpose Elderly patients with acute myeloid leukemia (AML) have a poor prognosis, and innovative maintenance therapy could improve their outcomes. Androgens, used in the treatment of aplastic anemia, have been reported to block proliferation of and initiate differentiation in AML cells. We report the results of a multicenter, phase III, randomized open-label trial exploring the benefit of adding androgens to maintenance therapy in patients 60 years of age or older. Patients and Methods A total of 330 patients with AML de novo or secondary to chemotherapy or radiotherapy were enrolled in the study...
February 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28128288/the-spleen-microenvironment-influences-disease-transformation-in-a-mouse-model-of-kit-d816v-dependent-myeloproliferative-neoplasm
#3
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/28107815/upfront-haploidentical-transplant-for-acquired-severe-aplastic-anemia-registry-based-comparison-with-matched-related-transplant
#4
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/28088971/-eltrombopag-for-refractory-thrombocytopenia-in-patients-with-allogeneic-hematopoietic-stem-cell-transplantation
#5
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/28081344/peripheral-flt-3-ligand-levels-as-a-pathobiological-parameter-duringthe-clinical-course-of-acute-myeloid-leukemia
#6
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/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
#7
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/27913465/aplastic-anemia-and-clonal-evolution-germ-line-and-somatic-genetics
#8
Akiko Shimamura
Clonal progression to myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) remains a dreaded complication for a subset of patients with bone marrow failure (BMF). Recognizing risk factors for the development of MDS or AML would inform individualized treatment decisions and identify patients who may benefit from early or upfront hematopoietic stem cell transplantation. Now that next-generation DNA sequencing is available in the clinical laboratory, research has focused on the implications of germ line and somatic mutations for diagnosing and monitoring patients with BMF...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27806787/-analysis-of-clinical-characteristics-and-prognosis-of-non-severe-aplastic-anemia-children-with-chromosomal-abnormalities
#9
S Zhu, W B An, Y Wan, Y Y Ren, R R Zhang, J L Zhang, C Liu, C C Sun, L X Chang, X J Chen, W Y Yang, Y Guo, Y M Chen, Y Zou, X F Zhu
Objective: To analyze the clinical characteristics and prognosis of non-severe aplastic anemia (NSAA) with chromosomal abnormalities in children. Method: A retrospective analysis of 304 cases with NSAA with successful karyotyping from 2001 to 2014 in the Institute of Hematology & Blood Disease Hospital was carried out. The treatment response, condition of blood transfusion were analyzed using χ(2) test, the cumulative survival was estimated by the Kaplan-Meier method. Result: Out of 304 patients, 28 patients had chromosomal abnormalities with trisomy 8 (7 cases, 25...
November 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27722132/changes-in-serum-uric-acid-levels-after-allogeneic-hematologic-stem-cell-transplantation-a-retrospective-cohort-study
#10
Sang Hyun Joo, Jin Kyun Park, Eunyoung Emily Lee, Yeong Wook Song, Sung-Soo Yoon
BACKGROUND: Since cell turnover in the hematopoietic system constitutes a major source of uric acid (UA) production, we investigated whether hematopoietic stem cell transplantation (HSCT) is associated with significant changes in serum UA levels in patients with hematological disorders. METHODS: Patients who underwent HSCT at our institution between 2001 and 2012 were retrospectively enrolled. Serum UA levels at 3 months before, 1 week before, and 3 months and 1 year after HSCT were examined...
September 2016: Blood Research
https://www.readbyqxmd.com/read/27693387/alterations-in-the-bone-marrow-microenvironment-may-elicit-defective-hematopoiesis-a-comparison-of-aplastic-anemia-chronic-myeloid-leukemia-and-normal-bone-marrow
#11
Meerim Park, Chan-Jeoung Park, Young Wook Cho, Seongsoo Jang, Jung-Hee Lee, Je-Hwan Lee, Kyoo-Hyung Lee, Young Ho Lee
Hematopoiesis involves complex interactions between hematopoietic cells and the bone marrow (BM) microenvironment. The specific causes and mechanisms underlying dysregulated hematopoiesis are unknown. Here, BM biopsy specimens from patients with aplastic anemia (AA) and chronic myeloid leukemia (CML) and normal marrow were analyzed by semiquantitative immunohistochemistry to determine changes in the hematopoietic stem cell (HSC) compartment and BM microenvironment. HSC levels were lowest in AA and highest in CML...
January 2017: Experimental Hematology
https://www.readbyqxmd.com/read/27634200/necroptosis-in-spontaneously-mutated-hematopoietic-cells-induces-autoimmune-bone-marrow-failure-in-mice
#12
Junping Xin, Peter Breslin, Wei Wei, Jing Li, Rafael Gutierrez, Joseph Cannova, Allen Ni, Grace Ng, Rachel Schmidt, Haiyan Chen, Vamsi Parini, Paul C Kuo, Ameet R Kini, Patrick Stiff, Jiang Zhu, Jiwang Zhang
Acquired aplastic anemia is an autoimmune-mediated bone marrow failure syndrome. The mechanism by which such an autoimmune reaction is initiated is unknown. Whether and how the genetic lesions detected in patients cause autoimmune bone marrow failure have not yet been determined. We found that mice with spontaneous deletion of the TGFβ-activated kinase-1 gene in a small subset of hematopoietic cells developed bone marrow failure which resembled the clinical manifestations of acquired aplastic anemia patients...
February 2017: Haematologica
https://www.readbyqxmd.com/read/27607445/hematopoietic-stem-cell-transplantation-for-acquired-aplastic-anemia
#13
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
https://www.readbyqxmd.com/read/27496498/autologous-hematopoietic-stem-cell-transplantation-for-adults-with-acute-myeloid-leukemia
#14
M Cioch, D Jawniak, M Wach, J Mańko, K Radomska, H Borowska, A Szczepanek, M Hus
BACKGROUND: Hematopoietic stem cell transplantation (HSCT) remains the most efficacious therapy in patients with acute leukemia. For older patients and those lacking a related HLA-compatible donor, autologous transplantation (auto-HSCT) is a valid alternative therapeutic option. METHODS: From 1997 until 2014 in the Department of Hematooncology and Bone Marrow Transplantation, Medical University of Lublin, Poland, 29 auto-HSCT were performed in patients with acute myeloid leukemia (AML; 15 men and 14 women; median age, 52...
June 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27455820/intracranial-hemorrhage-in-patients-with-hematologic-disorders-prevalence-and-predictive-factors
#15
Weerapat Owattanapanich, Chirayu U Auewarakul
BACKGROUND: Intracranial hemorrhage (ICH) is an uncommon complication in patients with hematologic disorders although high fatality rates have been shown in these patients. At present, no epidemiological data regarding ICH in patients with hematologic disorders has been collected and/or reported in Thailand. OBJECTIVE: The purpose of this study was to determine the incidence of ICH in hospitalized patients with hematologic disorders and to identify predictive factors associated with ICH in these patients...
January 2016: Journal of the Medical Association of Thailand, Chotmaihet Thangphaet
https://www.readbyqxmd.com/read/27408397/leukemic-transformation-of-severe-aplastic-anemia-following-matched-allogenic-stem-cell-transplantation-transplanted-again-in-cr-1
#16
Prabhu Manivannan, Abhishek Purohit, Venkatesan Somasundaram, Ankur Ahuja, Mukul Aggarwal, Rajiv Kumar, Pawan Kumar Singh, Hara Prasad Pati, Pravas Mishra, Tulika Seth, Manoranjan Mahapatra
Aplastic anemia (AA) is a life-threatening bone marrow failure disorder, if untreated, is associated with very high mortality. Allogenic bone marrow transplantation (BMT) is the standard of care for severe aplastic anemia (SAA) patients those who are younger than 40 years of age. The development of secondary malignancies in post-BMT setting for AA is a rare, however, well documented phenomenon. Among the secondary malignancies, development of acute myeloid leukemia is even rarer entity. Here we report a case of acute myeloid leukemia following human leucocyte antigen (HLA) matched sibling peripheral blood stem cell transplant (PBSCT) in a case of SAA...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27352174/haplo-identical-transplantation-for-acquired-severe-aplastic-anaemia-in-a-multicentre-prospective-study
#17
Lan-Ping Xu, Shun-Qing Wang, De-Pei Wu, Jian-Min Wang, Su-Jun Gao, Ming Jiang, Cun-Bang Wang, Xi Zhang, Qi-Fa Liu, Ling-Hui Xia, Xin Wang, Xiao-Jun Huang
We conducted a prospective, multicentre study to confirm the feasibility of haplo-identical transplantation in treatment of severe aplastic anaemia (SAA) as salvage therapy, by analysing the outcomes of 101 patients who received haplo-identical transplantation between June 2012 and October 2015. All cases surviving for more than 28 d achieved donor myeloid engraftment. The median time for myeloid engraftment was 12 (range, 9-25) days and 15 (range, 7-101) days for platelets, with a cumulative platelet engraftment incidence of 94·1 ± 0·1%...
October 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/27352093/causes-of-macrocytic-anemia-among-628-patients-mean-corpuscular-volumes-of-114-and-130%C3%A2-fl-as-critical-markers-for-categorization
#18
Natsuko Takahashi, Junichi Kameoka, Naoto Takahashi, Yoshiko Tamai, Kazunori Murai, Riko Honma, Hideyoshi Noji, Hisayuki Yokoyama, Yasuo Tomiya, Yuichi Kato, Kenichi Ishizawa, Shigeki Ito, Yoji Ishida, Kenichi Sawada, Hideo Harigae
There have been no studies on the distribution of causes of macrocytic anemia with respect to mean corpuscular volume (MCV) cutoff values. We retrospectively investigated the causes of macrocytic anemia (MCV ≥100 fL) among 628 patients who visited the outpatient hematology clinic in Tohoku University Hospital. To ensure data validity, we also analyzed data from 307 patients in eight other hospitals in the Tohoku district. The leading causes of macrocytic anemia (number of patients, %) were myelodysplastic syndromes (121, 19...
September 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27313707/development-of-donor-cell-leukemia-following-peripheral-blood-stem-cell-transplantation-for-severe-aplastic-anemia-a-case-report
#19
Hongbing Ma, Ting Liu
Donor cell leukemia (DCL) is a rare complication of hematopoietic stem cell transplantation (HSCT) which occurs in ~5% of all leukemic relapses. In the English literature, >60 cases of DCL have been reported, however, only two cases of DCL following HSCT for the treatment of severe aplastic anemia (SAA) have been described to date. In the present study, the case of a 25 year-old male patient diagnosed with SAA, who underwent a peripheral blood stem cell transplantation (PBSCT) using cells obtained from a sibling with an identical human leukocyte antigen, is presented...
June 2016: Oncology Letters
https://www.readbyqxmd.com/read/27283238/serum-metabolomics-of-acute-lymphoblastic-leukaemia-and-acute-myeloid-leukaemia-for-probing-biomarker-molecules
#20
Syed Ghulam Musharraf, Amna Jabbar Siddiqui, Tahir Shamsi, Arshi Naz
Acute leukaemia (AL) is a critical neoplasm of white blood cells. Diagnosing AL requires bone marrow puncture procedure, which many patients do not consent to for it is invasive. Hence sensitive and specific early diagnostic biomarkers are essential for non-invasive diagnosis, new therapeutics and improving the disease prognosis. To differentiate the metabolic alterations associated with acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML), we investigated serum of ALL and AML patients in comparison with two controls using gas chromatography coupled with triple quadrupole tandem mass spectrometry and multivariate statistical analysis...
June 10, 2016: Hematological Oncology
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