24,713 results match your criteria Myelodysplastic Syndrome


Pre-analytical parameters associated with unsuccessful karyotyping in myeloid neoplasm: a study of 421 samples.

Braz J Med Biol Res 2019 Feb 14;52(2):e8194. Epub 2019 Feb 14.

Hospital Israelita Albert Einstein, São Paulo, SP, Brasil.

Cytogenetics is essential in myeloid neoplasms (MN) and pre-analytical variables are important for karyotyping. We assessed the relationship between pre-analytical variables (time from collection to sample processing, material type, sample cellularity, and diagnosis) and failures of karyotyping. Bone marrow (BM, n=352) and peripheral blood (PB, n=69) samples were analyzed from acute myeloid leukemia (n=113), myelodysplastic syndromes (n=73), myelodysplastic syndromes/myeloproliferative neoplasms (n=17), myeloproliferative neoplasms (n=137), and other with conclusive diagnosis (n=6), and reactive disorders/no conclusive diagnosis (n=75). Read More

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http://dx.doi.org/10.1590/1414-431X20188194DOI Listing
February 2019

Complex karyotype AML displays G2/M signature and hypersensitivity to PLK1 inhibition.

Blood Adv 2019 Feb;3(4):552-563

The Leucegene Project at Institute for Research in Immunology and Cancer, Université de Montréal, Montréal, QC, Canada.

Patients diagnosed with acute myeloid leukemia with complex karyotype (CK AML) have an adverse prognosis using current therapies, especially when accompanied by alterations. We hereby report the RNA-sequencing analysis of the 68 CK AML samples included in the Leucegene 415 patient cohort. We confirm the frequent occurrence of alterations in this subgroup and further characterize the allele expression profile and transcript alterations of this gene. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018028480DOI Listing
February 2019

Correlation between IL-7 genomic protein methylation level and acute myeloid leukemia.

Authors:
Z-H Li Y Liu S-Y Gao

Eur Rev Med Pharmacol Sci 2019 Feb;23(3):1196-1202

Medicine Experimental Training Center, Weifang Medical University, Weifang, P. R. China.

Objective: To detect Interleukin-7 (IL-7) gene methylation status and transcription level in leukemia cells of peripheral blood of patients with Acute Myelocytic Leukemia (AML) and in the cell lines (HL-60, HL-60/ADM, SKM-1) of AML and myelodysplastic syndrome (MDS), and explore its relationship with the pathogenesis of AML.

Patients And Methods: A total of 55 AML patients (AML group) and 30 healthy adults (Healthy group) from June 2015 to June 2018 were enrolled in this study. The genomic DNA of leukemia cells in peripheral blood was extracted. Read More

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http://dx.doi.org/10.26355/eurrev_201902_17012DOI Listing
February 2019

The importance of adequate recognition of normal and dysplastic myelopoiesis for the diagnosis of myelodysplastic syndromes.

Histol Histopathol 2019 Feb 19:18093. Epub 2019 Feb 19.

Escola de Citologia Hematològica Soledad Woessner-Parc de Salut Mar. Laboratorio de Citología Hematológica. Patología. GRETNHE, IMIM Hospital del Mar Research Institute, Barcelona, Spain.

The diagnosis of myelodysplastic syndromes is based on the presence of cytopenias, dysplastic morphological features on peripheral blood (PB) and bone marrow (BM), cytogenetic abnormalities and requires to rule out other diseases resembling these conditions. Optical cytomorphology is the cornerstone of diagnosis of MDS. The recognition of cytological myelodysplasia has a crucial value in diagnosis and prognosis of MDS. Read More

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http://dx.doi.org/10.14670/HH-18-093DOI Listing
February 2019

Update of the GIMEMA MDS0306 study: deferasirox for lower risk transfusion-dependent patients with myelodysplastic syndromes.

Eur J Haematol 2019 Feb 18. Epub 2019 Feb 18.

Hematology and Bone Marrow Transplantation Unit, IRCCS Ospedale Policlinico San Martino, Genova.

Most patients affected by Myelodysplastic syndromes (MDS) develop transfusion dependence, which can be correlated with poorer outcomes partially caused by transfusional iron overload. Oral iron chelator deferasirox was approved in 2005 for the treatment of chronic iron overload due to blood transfusions, inclusive of transfusion-dependent lower IPSS risk (1-2). A prospective, open-label, single arm, Italian multicenter study (GIMEMA MDS0306) has been proposed to establish the safety and efficacy of deferasirox in a real-world population of transfusion-dependent lower IPSS MDS patients(3). Read More

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http://dx.doi.org/10.1111/ejh.13222DOI Listing
February 2019

A Myelodysplastic Syndrome with Concurrent Basophilia and Eosinophilia Lacking Oncogenic Mutations in 54 Relevant Genes.

Clin Lab 2019 Jan;65(1)

Myelodysplastic syndromes (MDS) with basophilia or eosinophilia are very rare and portend poor prognoses. We present a rare patient who had MDS with excess blasts as well as peripheral basophilia and concurrent bone marrow (BM) basophilia/eosinophilia. She had a complex karyotype including 5q and 7q deletions; however, no oncogenic mutations were observed on next-generation sequencing of 54 genes known to be frequently mutated in acute myeloid leukemia/MDS. Read More

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http://dx.doi.org/10.7754/Clin.Lab.2018.180729DOI Listing
January 2019

A phase 2 clinical trial of eltrombopag for treatment of patients with myelodysplastic syndromes after hypomethylating-agent failure.

Leuk Lymphoma 2019 Feb 18:1-7. Epub 2019 Feb 18.

a Department of Leukemia , University of Texas, MD Anderson Cancer Center , Houston , TX , USA.

Hypomethylating agents (HMA) are the standard of care for treatment of myelodysplastic syndromes (MDS). HMA-failure MDS has extremely poor prognosis. This study was designed to explore the utility of eltrombopag in post-HMA failure MDS patients. Read More

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http://dx.doi.org/10.1080/10428194.2019.1576873DOI Listing
February 2019

Somatic Mutation of HLA-DRB1*04:03 in a Patient with Myelodysplastic Syndrome at Diagnosis.

HLA 2019 Feb 17. Epub 2019 Feb 17.

Department of Laboratory Medicine, EwhaWomans University, College of Medicine, Seoul, South Korea.

Loss or decrease in expression of human leukocyte antigen (HLA) caused by somatic mutations of HLA genes has been reported in various malignancies. However, mutations in the HLA-DR gene have been rarely noted in hematologic malignancies. Here, we report a case of myelodysplastic syndrome (MDS) with a novel point mutation in exon 2 of the HLA-DRB1*04:03 gene pertaining to a silent mutation (c. Read More

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http://dx.doi.org/10.1111/tan.13490DOI Listing
February 2019

Flow cytometry diagnosis in myelodysplastic syndrome: Current practice in Latin America and comparison with other regions of the world.

Leuk Res 2019 Jan 24. Epub 2019 Jan 24.

Laboratorio de Citometria y Biologia Molecular, Departamento Basico de Medicina, Hospital de Clínicas, Facultad de Medicina, Universidad de la Republica, Montevideo, Uruguay. Electronic address:

Background: Flow cytometry (FC) is a valuable tool for the diagnosis of myelodysplastic syndromes (MDS). We present results of a survey carried out to evaluate FC current practice for MDS diagnosis in Latin America (LA), focusing on markers used and characteristics of the clinical diagnostic report. Compliance to IMDSflow recommendations was also evaluated. Read More

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http://dx.doi.org/10.1016/j.leukres.2019.01.009DOI Listing
January 2019

Therapeutic approaches to treat human spliceosomal diseases.

Curr Opin Biotechnol 2019 Feb 14;60:72-81. Epub 2019 Feb 14.

Department of Chemical and Biomolecular Engineering, University of California, Los Angeles, CA 90095, United States.

Mutated RNA splicing machinery drives many human diseases and is a promising therapeutic target for engineering and small molecule therapy. In the case of mutations in individual genes that cause them to be incorrectly spliced, engineered splicing factors can be introduced to correct splicing of these aberrant transcripts and reduce the effects of the disease phenotype. Mutations that occur in certain splicing factor genes themselves have been implicated in many cancers, particularly myelodysplastic syndromes. Read More

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http://dx.doi.org/10.1016/j.copbio.2019.01.003DOI Listing
February 2019
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F-FLT PET/MRI for bone marrow failure syndrome-initial experience.

EJNMMI Res 2019 Feb 15;9(1):16. Epub 2019 Feb 15.

Biomedical Imaging Research Center, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji-cho, Fukui, 910-1193, Japan.

Background: Bone marrow failure syndrome (BMFS) is a heterogeneous group of disorders associated with single- or multiple-lineage cytopenia and failure of normal hematopoiesis. We assessed the feasibility of integrated PET/MRI with 3'-deoxy-3'-F-fluorothymidine (F-FLT) to assess the pathophysiology of whole-body bone marrow for the diagnosis and monitoring of BMFS. Twenty-five consecutive patients with BMFS underwent a pre-treatment F-FLT PET/MRI scan. Read More

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http://dx.doi.org/10.1186/s13550-019-0490-0DOI Listing
February 2019
1 Read

LEF1-AS1, long non-coding RNA, inhibits proliferation in myeloid malignancy.

J Cell Mol Med 2019 Feb 15. Epub 2019 Feb 15.

Hematology and Hemotherapy Center, Hemocentro-Unicamp, São Paulo, Brazil.

LEF1 antisense RNA 1 (LEF1-AS1) is an antisense long non-coding RNA encoded in the lymphoid enhancer-binding factor 1 (LEF1) locus. LEF1-AS1 is a conserved transcript dysregulated in hematopoiesis. This study aimed to functionally characterize the role of this transcript in myeloid malignancy and explore a possible regulatory effect of LEF1-AS1 upon LEF1. Read More

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http://dx.doi.org/10.1111/jcmm.14152DOI Listing
February 2019
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Clinical Benefit-Risk Profile of Lenalidomide in Patients With Lower-risk Myelodysplastic Syndromes Without del(5q): Results of a Phase III Trial.

Clin Lymphoma Myeloma Leuk 2018 Dec 21. Epub 2018 Dec 21.

MDS Unit, AOU Careggi, University of Florence, Florence, Italy.

Background: In the phase III MDS-005 study of patients with lower-risk, non-del(5q) myelodysplastic syndromes, lenalidomide was associated with a higher rate of ≥ 8 weeks red blood cell transfusion independence (RBC-TI) compared with placebo, but also with a higher risk of hematologic adverse events (AEs).

Patients And Methods: This analysis evaluated the ratio of clinical benefit-risk in patients treated with lenalidomide or placebo, and assessed the effect of lenalidomide dose reductions on response. Clinical benefit was a composite endpoint defined as RBC-TI, transfusion reduction ≥ 4 units packed red blood cells, hemoglobin increase ≥ 1. Read More

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http://dx.doi.org/10.1016/j.clml.2018.12.012DOI Listing
December 2018
1 Read

Current Therapeutic Results and Treatment Options for Older Patients with Relapsed Acute Myeloid Leukemia.

Cancers (Basel) 2019 Feb 14;11(2). Epub 2019 Feb 14.

Division of Hematology, Spedali Civili, 25123 Brescia, Italy.

Considerable progress has been made in the treatment of acute myeloid leukemia (AML). However, current therapeutic results are still unsatisfactory in untreated high-risk patients and poorer in those with primary refractory or relapsed disease. In older patients, reluctance by clinicians to treat unfit patients, higher AML cell resistance related to more frequent adverse karyotype and/or precedent myelodysplastic syndrome, and preferential involvement of chemorefractory early hemopoietic precursors in the pathogenesis of the disease further account for poor prognosis, with median survival lower than six months. Read More

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http://dx.doi.org/10.3390/cancers11020224DOI Listing
February 2019
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BRD4 Inhibition Enhances Azacitidine Efficacy in Acute Myeloid Leukemia and Myelodysplastic Syndromes.

Front Oncol 2019 29;9:16. Epub 2019 Jan 29.

Hematology and Transfusion Medicine Center, Instituto Nacional de Ciência e Tecnologia do Sangue, University of Campinas, Hemocentro-Unicamp, São Paulo, Brazil.

Myelodysplastic syndromes (MDS) are clonal hematopoietic stem cell-based disorders characterized by ineffective hematopoiesis, increased genomic instability and a tendency to progress toward acute myeloid leukemia (AML). MDS and AML cells present genetic and epigenetic abnormalities and, due to the heterogeneity of these molecular alterations, the current treatment options remain unsatisfactory. Hypomethylating agents (HMA), especially azacitidine, are the mainstay of treatment for high-risk MDS patients and HMA are used in treating elderly AML. Read More

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http://dx.doi.org/10.3389/fonc.2019.00016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6361844PMC
January 2019
1 Read

Clonal Hematopoiesis with Oncogenic Potential (CHOP): Separation from CHIP and Roads to AML.

Int J Mol Sci 2019 Feb 12;20(3). Epub 2019 Feb 12.

Institute of Pathology, Ludwig-Maximilians University, 80539 Munich, Germany.

The development of leukemia is a step-wise process that is associated with molecular diversification and clonal selection of neoplastic stem cells. Depending on the number and combinations of lesions, one or more sub-clones expand/s after a variable latency period. Initial stages may develop early in life or later in adulthood and include premalignant (indolent) stages and the malignant phase, defined by an acute leukemia. Read More

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http://dx.doi.org/10.3390/ijms20030789DOI Listing
February 2019
6 Reads

Functional impairment of the HIPK2 small ubiquitin-like modifier (SUMO)-interacting motif in acute myeloid leukemia.

Am J Cancer Res 2019 1;9(1):94-107. Epub 2019 Jan 1.

Department of Biological Sciences, Sungkyunkwan University Suwon 16419, Republic of Korea.

Covalent conjugations of the SUMO-1 moiety on a target protein play important roles in the regulation of cellular protein function. SUMO-conjugation of PML is a regulatory step for PML nuclear body (PML-NB) formation, and HIPK2 is SUMO-conjugated and recruited into the PML-NBs. Although HIPK2 mutations (R861W and N951I) were found in acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) patients, little is known about the underlying mechanisms by which HIPK2 mutations are associated with the pathogenesis of leukemia. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6356924PMC
January 2019
1 Read

Anti-cancer effects of curcumin on myelodysplastic syndrome through the inhibition of enhancer of zeste homolog-2 (EZH2).

Curr Cancer Drug Targets 2019 Feb 12. Epub 2019 Feb 12.

Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030. China.

Enhancer of zeste homolog-2 (EZH2), a histone methyltransferase that regulates histone H3 methylation of lysine27 (H3K27me3), is involved in the pathogenesis of myelodysplastic syndrome (MDS). Targeting epigenetic regulators has been identified as a potential treatment target in MDS chemotherapy. Curcumin, a natural compound extracted from turmeric, was found to possess a wide range of anticancer activities in various tumors. Read More

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http://www.eurekaselect.com/169909/article
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http://dx.doi.org/10.2174/1568009619666190212121735DOI Listing
February 2019
3 Reads

Anti-CD117 antibody depletes normal and myelodysplastic syndrome human hematopoietic stem cells in xenografted mice.

Blood 2019 Feb 11. Epub 2019 Feb 11.

Department of Pediatrics, Division of Stem Cell Transplantation and Regenerative Medicine, Stanford University School of Medicine, Stanford, CA, United States

The myelodysplastic syndromes (MDS) represent a group of clonal disorders that result in ineffective hematopoiesis and are associated with an increased risk of transformation into acute leukemia. MDS arises from hematopoietic stem cells (HSCs); therefore, successful elimination of MDS HSCs is an important part of any curative therapy. However, current treatment options, including allogeneic hematopoietic cell transplantation (HCT), often fail to ablate disease-initiating MDS HSCs, and thus have low curative potential and high relapse rates. Read More

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http://dx.doi.org/10.1182/blood-2018-06-858159DOI Listing
February 2019
1 Read

[Consistency Analysis of Gene Mutation Sites of Bone Marrow Tumor DNA and Circulating Tumor DNA in Patients with Myelodysplastic Syndrome].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):149-158

Department of Hematology, Xiyuan Hospital, Academy of Chinese Medical Sciences, Beijing, 100091, China.E-mail:

Objective: To analyze the consistency of gene mutation sites between bone marrow DNA (BM-tDNA) and perepheral plasma circulating tumor DNA (PP-ctDNA) in patients with myelodysplastic syndrome (MDS).

Methods: The simultaneous sampled BM and PP from 19 patients (SBPP) was detected by NGS-127 gene panel, and the consistency of VAF between BM-tDNA and PP-ctDNA was analyzed. The peripheral blood cell tumor DNA (PC-tDNA) of 5 out of 19 patients was detected randomly, the consistency of VAF among PC-tDNA,BM-tDNA and PP-ctDNA was analyzed. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.024DOI Listing
February 2019
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[Application of Two-parameter Scoring System Based on CD105 and CD117 in MDS Diagnosis].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):141-148

Peking University People's Hospital,Peking University Institute of Hematology, Beijing 100044, China.E-mail:

Objective: To study the value of flow cytometric scoring system in the diagnosis of myelodysplastic syndromes (MDS).

Methods: The phenotypes of erythroid and immature cells were analyzed retrospectively in 130 MDS patients, 19 healthy controls and 89 pathological controls, all of them were well clinically immunophenotyped. The 4-parameter scoring system reported in the literature was studied, including myeloblast-related cluster size, B-progenitor-related cluster size, lymphocyte to myeloblast CD45 ratio, and granulocyte to lymphocyte side scatter ratio. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.023DOI Listing
February 2019
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[Expression and Function of miR-99a-5p in Bone Marrow of Patients with MDS].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2019 Feb;27(1):134-140

Department of Hematology, The First Affliated Hospital, Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China.E-mail:

Objective: To detect the expression of miR-99a-5p in myelodysplastic syndrome (MDS), to predict the target genes and to analyze its function by using bioinformatics.

Methods: The expression levels of bone marrow miR-99a-5p in MDS patients were detected by qRT-PCR, and the correlation of miR-99a-5p expression with clinical pathological characteristics, percentage of marrow blasts , chromosome karyotype and peripheral blood hemogram were analyzed. The target genes of miR-99a-5p were predicted by Targetscan, Miranda and Microcosm, and the intersection of the predicted results of 3 softwares was used as a potential target gene for miR-99a-5p. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2019.01.022DOI Listing
February 2019
1 Read

Residential ambient benzene exposure in the United States and subsequent risk of hematologic malignancies.

Int J Cancer 2019 Feb 9. Epub 2019 Feb 9.

Epidemiology Research Group, American Cancer Society, Atlanta, GA, USA.

Benzene is considered a carcinogen, mostly based on evidence of causality for myeloid leukemia from high levels of exposure in occupational studies. We used U.S. Read More

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http://dx.doi.org/10.1002/ijc.32202DOI Listing
February 2019
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S100A9-induced overexpression of PD-1/PD-L1 contributes to ineffective hematopoiesis in myelodysplastic syndromes.

Leukemia 2019 Feb 8. Epub 2019 Feb 8.

Department of Immunology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA.

Myelodysplastic syndromes (MDS) are characterized by dysplastic and ineffective hematopoiesis that can result from aberrant expansion and activation of myeloid-derived suppressor cells (MDSCs) within the bone marrow (BM) niche. MDSCs produce S100A9, which mediates premature death of hematopoietic stem and progenitor cells (HSPCs). The PD-1/PD-L1 immune checkpoint impairs immune responses by inducing T-cell exhaustion and apoptosis, but its role in MDS is uncharacterized. Read More

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http://dx.doi.org/10.1038/s41375-019-0397-9DOI Listing
February 2019
1 Read

A phase II study of guadecitabine in higher-risk myelodysplastic syndrome and low blast count acute myeloid leukemia after azacitidine failure.

Haematologica 2019 Feb 7. Epub 2019 Feb 7.

Hématologie clinique, Hôpital Saint-Louis, Paris, France;

High-risk myelodysplastic syndrome/acute myeloid leukemia patients have a very poor survival after azacitidine failure. Guadecitabine (SGI-110) is a novel subcutaneous hypomethylating agent, which results in extended decitabine exposure. This multicenter phase II study evaluated the efficacy and safety of guadecitabine in high-risk myelodysplastic syndrome and low blast count acute myeloid leukemia patients refractory or relapsing after azacitidine. Read More

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http://dx.doi.org/10.3324/haematol.2018.207118DOI Listing
February 2019
1 Read

Hematological Malignancies in Adults With a Family Predisposition.

Dtsch Arztebl Int 2018 12;115(50):848-854

Department of Internal Medicine V, Hematology, Oncology and Rheumatology, Heidelberg University Hospital and Clinical Cooperation Unit Molecular Hematology/Oncology, German Cancer Research Center (DKFZ) Heidelberg, Germany; Department of Internal Medicine V, Hematology, Oncology and Rheumatology, Heidelberg University Hospital, Heidelberg, Germany; Medical Oncology, National Center for Tumor Diseases (NCT), Heidelberg University Hospital, Heidelberg, Germany; Section Head of Translational Gynecology, University Women's Hospital Heidelberg, German Cancer Consortium (DKTK), Heidelberg, Germany; Institute of Pathology, Department of Applied Tumor Biology, Heidelberg University Hospital, Heidelberg, Germany.

Background: Some hematological malignancies arise in persons with a hereditary predisposition. The hereditary nature of these diseases often goes unrecognized, particularly when symptoms begin in adulthood.

Methods: This review is based on pertinent publications retrieved by a selective search in PubMed. Read More

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https://www.aerzteblatt.de/10.3238/arztebl.2018.0848
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http://dx.doi.org/10.3238/arztebl.2018.0848DOI Listing
December 2018
3 Reads

Using PU.1 and Jun dimerization protein 2 transcription factor expression in myelodysplastic syndromes to predict treatment response and leukaemia transformation.

Ann Hematol 2019 Feb 5. Epub 2019 Feb 5.

School of Life Sciences, College of Science, Joseph Banks Laboratories, University of Lincoln, Green Lane, Lincoln, Lincolnshire, LN6 7DL, UK.

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http://dx.doi.org/10.1007/s00277-019-03627-9DOI Listing
February 2019
1 Read

Iron Overload in Myelodysplastic Syndromes: Pathophysiology, Consequences, Diagnosis, and Treatment.

J Adv Pract Oncol 2018 May-Jun;9(4):392-405. Epub 2018 May 1.

University of Colorado, Aurora, Colorado.

Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic neoplasms varying in severity affecting one or more lines of hematopoiesis. Ineffective erythropoiesis results in dysregulation of iron metabolism. Most MDS patients have anemia, and some require regular red blood cell transfusions. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347085PMC
May 2018
1 Read

Clinical impact of the loss of chromosome 7q on outcomes of patients with myelodysplastic syndromes treated with allogeneic hematopoietic stem cell transplantation.

Bone Marrow Transplant 2019 Feb 4. Epub 2019 Feb 4.

Department of Hematology, Nagasaki University Hospital, Nagasaki, Japan.

We conducted a nationwide retrospective study to evaluate the prognostic influence of +1, der(1;7)(q10;p10) [hereafter der(1;7)] and -7/del(7q) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) for de novo myelodysplastic syndromes (MDS). In this database, 69 MDS patients with der(1;7), 75 with -7/del(7q), and 511 with normal karyotype (NK) underwent allo-HSCT at advanced disease status. The 3-year overall survival (OS) and cumulative incidence of relapse (CIR) were 50. Read More

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http://dx.doi.org/10.1038/s41409-019-0469-5DOI Listing
February 2019
2 Reads

Incidence, risk factors, and clinical significance of Epstein-Barr virus reactivation in myelodysplastic syndrome after allogeneic haematopoietic stem cell transplantation.

Ann Hematol 2019 Feb 4. Epub 2019 Feb 4.

Jiangsu Institute of Haematology, The First Affiliated Hospital of Soochow University, Suzhou, China.

Epstein-Barr virus (EBV) reactivation is a life-threatening complication after allogeneic haematopoietic stem cell transplantation (allo-HSCT). In this study, we investigated the characteristics of EBV reactivation in 186 consecutive myelodysplastic (MDS) patients who underwent allo-HSCT in our centre. In 35 patients (18. Read More

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http://link.springer.com/10.1007/s00277-019-03603-3
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http://dx.doi.org/10.1007/s00277-019-03603-3DOI Listing
February 2019
2 Reads

Monozygotic twins with shared de novo GATA2 mutation but dissimilar phenotypes due to differential promoter methylation.

Leuk Lymphoma 2019 Feb 4:1-9. Epub 2019 Feb 4.

a Department of Laboratory Medicine , Seoul National University College of Medicine , Seoul , Republic of Korea.

A revised WHO classification of hematopoietic neoplasm introduced the new category 'Myeloid Neoplasms with Germline Predisposition', reflecting the growing importance of genetic testing for myeloid neoplasms. Here, we investigated monozygotic twins with the same de novo mutation in GATA2 but different phenotypes. The patient suffering a bleeding tendency was diagnosed with myelodysplastic syndrome (MDS), and her monozygotic twin showed dysmegakaryopoietic features in the bone marrow. Read More

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http://dx.doi.org/10.1080/10428194.2018.1516039DOI Listing
February 2019
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A Phase II Study to Determine the Safety and Efficacy of the Oral Inhibitor of Indoleamine 2,3-Dioxygenase (IDO) Enzyme INCB024360 in Patients with Myelodysplastic Syndromes.

Clin Lymphoma Myeloma Leuk 2018 Dec 20. Epub 2018 Dec 20.

MD Moffitt Cancer Center and Research Institute, Tampa, FL.

Background: INCB024360 is an oral inhibitor of the enzyme indoleamine 2,3-dioxygenase (IDO), which catalyzes the degradation of tryptophan to kynurenine. Preclinical data suggest that IDO1 inhibition by INCB024360 will increase T cell proliferation, and decrease T regulatory cells and myeloid derived suppressor cells suppressive activity. We conducted a phase II study to explore activity and pharmacodynamics of INCB024360 in patients with myelodysplastic syndromes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S21522650183119
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http://dx.doi.org/10.1016/j.clml.2018.12.005DOI Listing
December 2018
2 Reads

Invasive aspergillosis manifesting as retinal necrosis in a patient treated with ruxolitinib.

Arch Soc Esp Oftalmol 2019 Jan 31. Epub 2019 Jan 31.

Sección de Retina Médica, Departamento de Oftalmología, Hospital Universitario Virgen del Rocío, Sevilla, España.

A 30 year-old man with acute myeloblastic leukaemia and secondary myelodysplastic syndrome developed graft-versus-host disease. The patient was treated with ruxolitinib. After being treated for 3 months with ruxolitinib, an inhibitor of Janus kinase, he developed Aspergillus retinal necrosis resistant to common treatment. Read More

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http://dx.doi.org/10.1016/j.oftal.2018.12.006DOI Listing
January 2019
1 Read

Pyoderma gangrenosum: a presenting sign of myelodysplastic syndrome in undiagnosed Fanconi anemia.

Dermatol Online J 2019 Jan 15;25(1). Epub 2019 Jan 15.

Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana.

A 26-year-old man with a history of congenital bilateral microtia, unilateral renal agenesis, left aural atresia, and right external auditory canal occlusion admitted for right rib cartilage graft harvest and left ear re-construction. Following surgery, an ulceration with violaceous borders and a yellow fibrinous base unresponsive to broad-spectrum antibiotics developed at the harvest site. The wound was expanding and not responsive to systemic broad-spectrum antibiotics. Read More

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January 2019
2 Reads

GATA2 hypomorphism induces chronic myelomonocytic leukemia in mice.

Cancer Sci 2019 Feb 2. Epub 2019 Feb 2.

Department of Molecular Hematology, Tohoku University Graduate School of Medicine, Sendai, Japan.

The transcription factor GATA2 regulates normal hematopoiesis, particularly in stem cell maintenance and myeloid differentiation. Various heteroallelic GATA2 gene mutations are associated with a variety of hematological neoplasms, including myelodysplastic syndromes and leukemias. Here, we report that impaired GATA2 expression induces myelodysplastic and myeloproliferative neoplasm development in elderly animals, and this neoplasm resembles chronic myelomonocytic leukemia in humans. Read More

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http://doi.wiley.com/10.1111/cas.13959
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http://dx.doi.org/10.1111/cas.13959DOI Listing
February 2019
13 Reads

Hematologic malignancies and Li-Fraumeni syndrome.

Cold Spring Harb Mol Case Stud 2019 Feb 1;5(1). Epub 2019 Feb 1.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas 77005, USA.

Li-Fraumeni syndrome (LFS) is an autosomal dominant condition associated with a high risk of a broad range of childhood- and adult-onset cancers. LFS is related to germline mutations of the tumor-suppressor gene The most common reported leukemia associated with LFS is hypodiploid acute lymphoblastic leukemia, but myeloid malignancies including acute myeloid leukemia (AML), chronic myeloid leukemia, and myelodysplastic syndrome (MDS) are also reported, often in the setting of therapy-related disease. We reviewed the clinicopathologic characteristics including cytogenetics and molecular analysis for seven adult patients with LFS and hematologic malignancies evaluated at the Hereditary Hematologic Malignancy Clinic (HHMC) at MD Anderson Cancer Center. Read More

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http://molecularcasestudies.cshlp.org/lookup/doi/10.1101/mcs
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http://dx.doi.org/10.1101/mcs.a003210DOI Listing
February 2019
10 Reads

α-Lipoic Acid Reduces Iron-induced Toxicity and Oxidative Stress in a Model of Iron Overload.

Int J Mol Sci 2019 Jan 31;20(3). Epub 2019 Jan 31.

Department of Medical, Surgical Sciences and Advanced Technologies "GF Ingrassia", University of Catania, 95123 Catania, Italy.

Iron toxicity is associated with organ injury and has been reported in various clinical conditions, such as hemochromatosis, thalassemia major, and myelodysplastic syndromes. Therefore, iron chelation therapy represents a pivotal therapy for these patients during their lifetime. The aim of the present study was to assess the iron chelating properties of α-lipoic acid (ALA) and how such an effect impacts on iron overload mediated toxicity. Read More

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http://dx.doi.org/10.3390/ijms20030609DOI Listing
January 2019
2 Reads
2.862 Impact Factor

Post-allogeneic eculizumab as prophylaxis against hemolysis and thrombosis for patients with hematologic disorders associated with PNH clones.

Biol Blood Marrow Transplant 2019 Jan 29. Epub 2019 Jan 29.

Department of Hematology and Hematopoietic Cell Transplantation, City of Hope, Duarte, CA; Department of Hematology and Hematopoietic Cell Transplantation, Gehr Family Center for Leukemia Research, City of Hope, Duarte, CA.

Paroxysmal nocturnal hemoglobinuria (PNH) is frequently seen in the context of other aplastic anemia and myelodysplastic syndrome and is associated with hemolysis and increased thromboembolic events. Allogeneic hematopoietic stem cell transplantation (alloHCT) is the only curative treatment but is associated with significant morbidity. The terminal complement inhibitor eculizumab reduces hemolysis and thromboembolic events and is the only FDA-approved therapy for PNH. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.01.025DOI Listing
January 2019
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Transcription factor mutations as a cause of familial myeloid neoplasms.

J Clin Invest 2019 Feb 1;129(2):476-488. Epub 2019 Feb 1.

UW-Madison Blood Research Program, Department of Cell and Regenerative Biology, Wisconsin Institutes for Medical Research, UW Carbone Cancer Center, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.

The initiation and evolution of myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) are driven by genomic events that disrupt multiple genes controlling hematopoiesis. Human genetic studies have discovered germline mutations in single genes that instigate familial MDS/AML. The best understood of these genes encode transcription factors, such as GATA-2, RUNX1, ETV6, and C/EBPα, which establish and maintain genetic networks governing the genesis and function of blood stem and progenitor cells. Read More

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https://www.jci.org/articles/view/120854
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http://dx.doi.org/10.1172/JCI120854DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6355228PMC
February 2019
4 Reads

Pediatric acute myeloid leukemia with t(7;21)(p22;q22).

Genes Chromosomes Cancer 2019 Jan 31. Epub 2019 Jan 31.

Department of Pathology, University of Utah, Salt Lake City, Utah.

The t(7;21)(p22;q22) resulting in RUNX1-USP42 fusion, is a rare but recurrent cytogenetic abnormality associated with acute myeloid leukemia (AML) and myelodysplastic syndromes. The prognostic significance of this translocation has not been well established due to the limited number of patients. Herein, we report three pediatric AML patients with t(7;21)(p22;q22). Read More

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http://doi.wiley.com/10.1002/gcc.22740
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http://dx.doi.org/10.1002/gcc.22740DOI Listing
January 2019
6 Reads

The prognostic value of circulating myeloblasts in patients with myelodysplastic syndromes treated with azacitidine.

Med Oncol 2019 Jan 31;36(3):25. Epub 2019 Jan 31.

Department of Hematology, Yokohama Municipal Citizen's Hospital, 56 Okazawa-cho, Hodogaya-ku, Yokohama, 240-8555, Japan.

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http://dx.doi.org/10.1007/s12032-019-1247-3DOI Listing
January 2019
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Tracking of myeloid malignancies by targeted analysis of successive DNA methylation at neighboring CG dinucleotides.

Haematologica 2019 Jan 31. Epub 2019 Jan 31.

Helmholtz-Institute for Biomedical Engineering, RWTH Aachen University, Aachen, Germany;

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http://dx.doi.org/10.3324/haematol.2018.209734DOI Listing
January 2019
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Disruption of erythroid nuclear opening and histone release in myelodysplastic syndromes.

Cancer Med 2019 Jan 30. Epub 2019 Jan 30.

Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois.

Mammalian terminal erythropoiesis involves several characteristic phenomena including chromatin condensation and enucleation. One of the newly identified features of terminal erythropoiesis in mouse is a dynamic nuclear opening and histone release process, which is required for chromatin condensation. However, it is unclear whether the same feature is present in human. Read More

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http://dx.doi.org/10.1002/cam4.1969DOI Listing
January 2019
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Central nervous system varicella zoster vasculopathy in an immunocompromised patient.

IDCases 2019 29;15:e00483. Epub 2018 Dec 29.

Infectious Diseases Department, Centro Hospitalar Universitário São João, Oporto, Portugal.

Central nervous system (CNS) vasculopathy associated with Varicella Zoster Virus (VZV) infection, usually manifesting as stroke due to ischemic lesions by involvement of small arteries, is frequently misdiagnosed. Immunocompromised patients have a particularly higher risk of severe disease and also CNS involvement during or following VZV presentations. We report a case of an 84-year-old man, with myelodysplastic syndrome, who presented with herpes zoster ophthalmicus complicated with left periocular cellulitis and an abnormal neurological exam. Read More

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http://dx.doi.org/10.1016/j.idcr.2018.e00483DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348231PMC
December 2018
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Massive Hemoptysis Due to the Rupture of Thoracic Aortic Aneurysm Caused by Leukemic Cell Infiltration in a Patient With Chronic Myelomonocytic Leukemia.

J Clin Med Res 2019 Feb 5;11(2):145-150. Epub 2019 Jan 5.

Department of Clinical Pathology, Asahi General Hospital, Chiba, Japan.

Hemoptysis is occasionally experienced in patients with hematological malignancies who have respiratory tract infection and severe thrombocytopenia. Thrombocytopenia due to hematological disease is one cause of hemoptysis. Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy characterized by both a myeloproliferative neoplasm and a myelodysplastic syndrome. Read More

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http://dx.doi.org/10.14740/jocmr3712DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340676PMC
February 2019
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Rethinking clinical trial endpoints in myelodysplastic syndromes.

Leukemia 2019 Jan 30. Epub 2019 Jan 30.

Leukemia Programs, Cleveland Clinic Taussig Cancer Institute and Dana-Farber Cancer Institute, Cleveland, OH, and Boston, MA, USA.

The myelodysplastic syndromes (MDS) are a heterogeneous collection of clonal, hematopoietic disorders primarily affecting an older population, making successful drug development a complicated process. A sole focus on response rate in clinical trials is likely not clinically meaningful if not accompanied by substantive response duration, improvement in quality of life, and ideally prolongation of survival. The process of receiving a new therapy should not be more burdensome than the MDS sequela it is intended to ameliorate. Read More

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http://dx.doi.org/10.1038/s41375-018-0367-7DOI Listing
January 2019
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The NUP98-HOXD13 fusion oncogene induces thymocyte self-renewal via Lmo2/Lyl1.

Leukemia 2019 Jan 30. Epub 2019 Jan 30.

Australian Centre for Blood Diseases, Monash University, Melbourne, VIC, Australia.

T cell acute lymphoblastic leukaemia (T-ALL) cases include subfamilies that overexpress the TAL1/LMO, TLX1/3 and HOXA transcription factor oncogenes. While it has been shown that TAL1/LMO transcription factors induce self-renewal of thymocytes, whether this is true for other transcription factor oncogenes is unknown. To address this, we have studied NUP98-HOXD13-transgenic (NHD13-Tg) mice, which overexpress HOXA transcription factors throughout haematopoiesis and develop both myelodysplastic syndrome (MDS) progressing to acute myeloid leukaemia (AML) as well as T-ALL. Read More

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http://www.nature.com/articles/s41375-018-0361-0
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http://dx.doi.org/10.1038/s41375-018-0361-0DOI Listing
January 2019
5 Reads