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Myeloid aplastic

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
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), 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...
September 27, 2016: Experimental Hematology
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...
September 15, 2016: Haematologica
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
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
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
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
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%...
June 28, 2016: British Journal of Haematology
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
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
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
Sebastian Fernandez-Pol, Lisa Ma, Robert S Ohgami, Daniel A Arber
In this study, we set out to evaluate the frequency of mutations in 20 myelodysplastic syndrome-associated genes in 53 individuals with pancytopenia in which bone marrow evaluation failed to meet standard criteria for a diagnosis of myelodysplastic syndrome. These idiopathic pancytopenia cases were associated with no specific cause for their pancytopenia (n=28), aplastic anemia (n=13), pancytopenia attributable to liver disease (n=4), pancytopenia associated with autoimmune disease (n=4), and pancytopenia attributed to drug effect (n=4)...
September 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Tetsuichi Yoshizato
Acquired aplastic anemia (AA) is a prototype of idiopathic bone marrow failure, which is caused by immune-mediated destruction of hematopoietic progenitors but is also characterized by frequent evolution to clonal myeloid disorders, such as myelodysplastic syndromes or acute myeloid leukemia. However, the chronological behavior of the clonality and its link to myelodysplastic syndrome or acute myeloid leukemia has not been fully explored. To define the clonality and its chronological behavior in AA, we performed targeted sequencing (N=439) in cases with AA...
April 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Seishi Ogawa
Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the advent of high-throughput sequencing of recent years, the molecular aspect of CH in AA has been clarified by comprehensive detection of somatic mutations that drive clonal evolution. Genetic abnormalities are found in ∼50% of patients with AA and, except for PIGA mutations and copy-neutral loss-of-heterozygosity, or uniparental disomy (UPD) in 6p (6pUPD), are most frequently represented by mutations involving genes commonly mutated in myeloid malignancies, including DNMT3A, ASXL1, and BCOR/BCORL1 Mutations exhibit distinct chronological profiles and clinical impacts...
July 21, 2016: Blood
Sarita Pradhan
A 61years old female presented with decreasing hemoglobin for past 6 months. She had a history of multiple transfusions in the recent past. Laboratory investigations showed: hemoglobin of 8.6 g/dL, total blood leukocyte count of 1.13 x109/L, and platelets of 80x109/L with presence of occasional circulating blasts. Bone marrow examination revealed presence of 63% myeloblasts with prominent Auer rods and mild reactive plasmacytosis(6%). Some of the plasma cells showed Auer rod like thin slender inclusions. She was diagnosed as Acute Myeloid Leukemia...
April 18, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Young Hoon Park, Joo Han Lim, Hyeon Gyu Yi, Moon Hee Lee, Chul Soo Kim
: BK virus-hemorrhagic cystitis (BKV-HC) is a potential cause of morbidity and mortality in patients having undergone allogeneic stem cell transplantation (Allo-SCT). We analyzed the clinical features of BKV-HC following Allo-SCT and reported the utility of leflunomide therapy for BKV-HC. MATERIALS AND METHODS: From January 2005 to June 2014, among the 69 patients underwent Allo-SCT in our institution, the patients who experienced BKV-HC were investigated retrospectively...
April 18, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
J C W Marsh, G J Mufti
Aplastic anaemia (AA) is frequently associated with other disorders of clonal haemopoiesis such as paroxysmal nocturnal haemoglobinuria (PNH), myelodysplastic syndrome (MDS) and T-large granular lymphocytosis. Certain clones may escape the immune attack within the bone marrow environment and proliferate and attain a survival advantage over normal haemopoietic stem cells, such as trisomy 8, loss of heterozygosity of short arm of chromosome 6 and del13q clones. Recently acquired somatic mutations (SM), excluding PNH clones, have been reported in around 20-25 % of patients with AA, which predispose to a higher risk of later malignant transformation to MDS/acute myeloid leukaemia...
August 2016: International Journal of Hematology
Serdar Sivgin, Esen Karakus, Muzaffer Keklik, Gokmen Zararsiz, Musa Solmaz, Leylagul Kaynar, Bulent Eser, Mustafa Cetin, Ali Unal
OBJECTIVES AND AIM: In this study, we aimed to compare the potency of different G-CSF agents including original filgrastim (Neupogen®), biosimilar filgrastim (Leucostim®) and Lenograstim (Granocyte®) on CD34(+) cell mobilization in patients that underwent allogeneic hematopoietic stem cell transplantation (alloHSCT). PATIENTS AND METHODS: The data of 243 donors for alloHSCT recipients diagnosed with mostly acute leukemia and myelodsyplastic syndromes (MDS) were analyzed, retrospectively...
June 2016: Transfusion and Apheresis Science
Erden Atilla, Pervin Topcuoglu, Pinar Ataca, Gultekin Pekcan, Sinem Civriz Bozdag, Meltem Kurt Yuksel, Muhit Ozcan, Gunhan Gurman, Selami Kocak Toprak
Post-transplant erythrocytosis is an infrequent complication and has been reported after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in aplastic anemia, acute myeloid leukemia, and chronic myeloid leukemia. The pre-disposing factors and treatment are not clearly defined. We present 11 post-transplant erythrocytosis cases. More studies should be conducted to distinguish the pathogenesis and follow-up for this rare complication.
June 2016: Clinical Transplantation
Michaela Nováková, Markéta Žaliová, Martina Suková, Marcin Wlodarski, Aleš Janda, Eva Froňková, Vít Campr, Kateřina Lejhancová, Ondřej Zapletal, Dagmar Pospíšilová, Zdeňka Černá, Tomáš Kuhn, Peter Švec, Vendula Pelková, Zuzana Zemanová, Gitte Kerndrup, Marry van den Heuvel-Eibrink, Vincent van der Velden, Charlotte Niemeyer, Tomáš Kalina, Jan Trka, Jan Starý, Ondřej Hrušák, Ester Mejstříková
GATA-2 deficiency was recently described as common cause of overlapping syndromes of immunodeficiency, lymphedema, familiar myelodysplastic syndrome or acute myeloid leukemia. The aim of our study was to analyze bone marrow and peripheral blood samples of children with myelodysplastic syndrome or aplastic anemia to define prevalence of the GATA2 mutation and to assess whether mutations in GATA-2 transcription factor exhibit specific immunophenotypic features. The prevalence of a GATA2 mutation in a consecutively diagnosed cohort of children was 14% in advanced forms of myelodysplastic syndrome (refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and myelodysplasia-related acute myeloid leukemia), 17% in refractory cytopenia of childhood, and 0% in aplastic anemia...
June 2016: Haematologica
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