19,566 results match your criteria Myelodysplastic Syndromes Unclassifiable


Unexplained cytopenias in an adolescent? You GATA think about it.

J Assoc Genet Technol 2018 ;44(4):135-136

Department of Pathology and Laboratory Medicine, University of Vermont Medical Center, Burlington, VT.

Objectives: The GATA family of DNA binding proteins consists of six different transcription factors (GATA1-6), each with a diverse biologic function. The transcription factors GATA1-3 function primarily to orchestrate hematopoiesis; however, they have roles in non-hematopoietic cells as well. Much of our current knowledge of the GATA transcription factors has come through observation of disease states with known GATA mutations. Read More

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January 2018

Benzene Exposure Response and Risk of Myeloid Neoplasms in Chinese Workers: A Multicenter Case-Cohort Study.

J Natl Cancer Inst 2018 Dec 6. Epub 2018 Dec 6.

Nanchang Center for Disease Control and Prevention, Nanchang, China.

Background: There is international consensus that benzene exposure is causally related to acute myeloid leukemia (AML), and more recent evidence of association with myelodysplastic syndromes (MDS). However, there are uncertainties about the exposure response, particularly risks by time since exposure and age at exposure.

Methods: In a case-cohort study in 110 631 Chinese workers followed up during 1972-1999 we evaluated combined MDS/AML (n = 44) and chronic myeloid leukemia (n = 18). Read More

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December 2018
2 Reads

The Rising Era of Immune Checkpoint Inhibitors in Myelodysplastic Syndromes.

Adv Hematol 2018 1;2018:2458679. Epub 2018 Nov 1.

Medical University of Varna, Varna, Bulgaria.

Myelodysplastic syndromes (MDS) are a heterogeneous group of diseases characterized by ineffective hematopoiesis and a wide spectrum of manifestations ranging from indolent and asymptomatic cytopenias to acute myeloid leukemia (AML). MDS result from genetic and epigenetic derangements in clonal cells and their surrounding microenvironments. Studies have shown associations between MDS and other autoimmune diseases. Read More

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November 2018

Direct modulation of the bone marrow mesenchymal stromal cell compartment by azacitidine enhances healthy hematopoiesis.

Blood Adv 2018 Dec;2(23):3447-3461

Department of Medicine III, Technische Universität München, Munich, Germany.

Mesenchymal stromal cells (MSCs) are crucial components of the bone marrow (BM) microenvironment essential for regulating self-renewal, survival, and differentiation of hematopoietic stem/progenitor cells (HSPCs) in the stem cell niche. MSCs are functionally altered in myelodysplastic syndromes (MDS) and exhibit an altered methylome compared with MSCs from healthy controls, thus contributing to disease progression. To determine whether MSCs are amenable to epigenetic therapy and if this affects their function, we examined growth, differentiation, and HSPC-supporting capacity of ex vivo-expanded MSCs from MDS patients in comparison with age-matched healthy controls after direct treatment in vitro with the hypomethylating agent azacitidine (AZA). Read More

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December 2018
1 Read

Long-term safety and activity of axicabtagene ciloleucel in refractory large B-cell lymphoma (ZUMA-1): a single-arm, multicentre, phase 1-2 trial.

Lancet Oncol 2018 Nov 30. Epub 2018 Nov 30.

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

Background: Axicabtagene ciloleucel is an autologous anti-CD19 chimeric antigen receptor (CAR) T-cell therapy. In the previous analysis of the ZUMA-1 registrational study, with a median follow-up of 15·4 months (IQR 13·7-17·3), 89 (82%) of 108 assessable patients with refractory large B-cell lymphoma treated with axicabtagene ciloleucel achieved an objective response, and complete responses were noted in 63 (58%) patients. Here we report long-term activity and safety outcomes of the ZUMA-1 study. Read More

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November 2018

A brief, but comprehensive, guide to clonal evolution in aplastic anemia.

Authors:
Daria V Babushok

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):457-466

Division of Hematology-Oncology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA; and.

Acquired aplastic anemia (AA) is an immune-mediated bone marrow aplasia that is strongly associated with clonal hematopoiesis upon marrow recovery. More than 70% of AA patients develop somatic mutations in their hematopoietic cells. In contrast to other conditions linked to clonal hematopoiesis, such as myelodysplastic syndrome (MDS) or clonal hematopoiesis of indeterminate potential in the elderly, the top alterations in AA are closely related to its immune pathogenesis. Read More

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November 2018
1 Read

Treatments targeting MDS genetics: a fool's errand?

Authors:
Amy E DeZern

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):277-285

Division of Hematologic Malignancies, John Hopkins Medicine, Baltimore, MD.

The myelodysplastic syndromes are collectively the most common myeloid neoplasms. Clonal hematopoiesis present in these diseases results in bone marrow failure characteristically seen in patients. The heterogeneity of myelodysplastic syndrome pathobiology has historically posed a challenge to the development of newer therapies. Read More

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November 2018

The MDS genomics-prognosis symbiosis.

Authors:
Aziz Nazha

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):270-276

Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH.

Myelodysplastic syndromes (MDS) are clonal disorders characterized by the accumulation of complex genomic abnormalities that define disease phenotype, prognosis, and the risk of transformation to acute myeloid leukemia. The clinical manifestations and overall outcomes of MDS are very heterogeneous with an overall survival that can be measured in years for some patients to a few months for others. Prognostic scoring systems are important staging tools that aid physicians in their treatment recommendations and decision-making and can help patients understand their disease trajectory and expectations. Read More

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November 2018

Successful treatment of invasive pulmonary aspergillosis caused by Aspergillus felis, a cryptic species within the Aspergillus section Fumigati: A case report.

J Infect Chemother 2018 Nov 30. Epub 2018 Nov 30.

Department of Infection Control Science, Osaka City University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan; Research Center for Infectious Disease Sciences (RCIDS), Osaka City University Graduate School of Medicine, 1-4-3, Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan. Electronic address:

Aspergillus species are a major cause of life-threatening infections in immunocompromised hosts, and the most common pathogen of invasive aspergillosis is Aspergillus fumigatus. Recently, the development of molecular identification has revealed cryptic Aspergillus species, and A. felis is one such species within the Aspergillus section Fumigati reported in 2013. Read More

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November 2018

Increased cardiovascular comorbidities in patients with myelodysplastic syndromes and chronic myelomonocytic leukemia presenting with systemic inflammatory and autoimmune manifestations.

Semin Hematol 2018 Oct 28;55(4):242-247. Epub 2018 May 28.

Department of Haematology and Central Haematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Switzerland; Department for BioMedical Research, Inselspital, Bern University Hospital, University of Bern, Switzerland. Electronic address:

Myelodysplastic syndromes (MDS) and associated diseases, like chronic myelomonocytic leukemias (CMML), are heterogeneous, clonal disorders affecting the hematopoietic stem cells. They are characterized by dysplasia and a propensity to evolve toward acute myeloid leukemia. Systemic inflammatory and autoimmune manifestations (SIAMs) occur with a prevalence of 10% to 20% in myeloid malignancies, but the underlying pathogenetic mechanisms remain obscure. Read More

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October 2018

Bone Marrow Fibrosis and Early Hematological Response as Predictors of Poor Outcome in Azacitidine Treated High Risk-Patients With Myelodysplastic Syndromes or Acute Myeloid Leukemia.

Semin Hematol 2018 Oct 21;55(4):202-208. Epub 2018 Feb 21.

Hematology department, Hematology Unit, IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan, Milan, Italy.

Azacitidine (AZA) treatment is effective treatment for patients with myeloid disorders, and factors predictive of treatment outcome are under investigation. Little is known about the effect of bone marrow fibrosis on response to AZA therapy. We, retrospectively, evaluated clinical predictors of overall survival (OS) and overall response rate (ORR) for patients treated with AZA in a real-life cohort. Read More

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October 2018

[Effects of Abnormal Iron Metabolism on EPO-STAT5 Signaling Pathway in Anemia Patients].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1726-1730

Department of Hematology, East Hospital of The Sixth People's Hospital, Shanghai Jiaotong University, Shanghai 201306,

Objective: To study the effects of iron metabolism abnormality on EPO-STAT5 signaling pathway in anemia patients.

Methods: According to diseases, the patients were divided into 3 groups: lower risk myelodysplastic syndrome (MDS) group (30 cases) including 14 cases of non-iron over load and 16 cases of iron over load, 12 cases of them were treated by iron chelation therapy; anemia of chronic disease (ACD) group (12 cases) and iron deficiency anemia (IDA) group (12 cases). In addition, the healthy control group was selected. Read More

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December 2018

[Predicting Therapeutic Response by Lymphocyte Level at 28th Day after DAC Treatment in Patients with MDS].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1719-1725

Department of Hematology, The Afilliatied Hospital of Hebei Universtity,Baoding 071000, Hebei Province, China.E-mail:

Objective: To investigate a reliable clinical indication for predicting the therapeutic response of decitabine therapy in the patients with myelodysplastic syndromes (MDS).

Methods: The clinical efficacy of decitabine for 55 cases of MDS was analyzed retrospectively. According to the lymphocyte level at d28 after the first time treatment with decitabine, the patients were divided into high lymphocyte level group (H-Lym≥1. Read More

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December 2018

[Correlation of the Serum bFGF Level with MDS Typing, Serum IL-32 Level and Prognosis].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1708-1712

Department of Hematology, The Fourth Hospital of Wuhan, Wuhan 430030, Hubei Province, China.E-mail:

Objective: To investigate the correlation of serum bFGF level with MDS typing, serum IL-32 and prognosis.

Methods: A total of 62 patients with myelodysplastic syndromes admitted in our hospital from April of 2014 to April of 2017 were enrolled in MDS group. And 50 healthy people who received healthy physical examination in our hospital were selected as the control group at the same period. Read More

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December 2018

[Clinical Efficacy and Prognostic Factors of Decitabine for Treatment of Myelodysplastic Syndrome].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1702-1707

Department of Hematology, Haiyang Municipal People's Hospital, Haiyang 265100, Shandong Province, China.

Objective: To explore the clinical efficacy of decitabine for treatment of patients with myelodysplastic syndrome (MDS) and factors predicting the prognosis.

Methods: The clinical data of 87 patients with MDS treated with decitabine were analyzed retrospectively. The hENT1 mRNA expression and TP53 gene mutation were detected by Q-PCR and gene target sequencing, respectively. Read More

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December 2018

[Clinical Charcteristics and Prognostic Analysis of 28 cases of Pediatric Myelodysplastic Syndrome].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1695-1701

Department of Pediatrics, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou 510120, Guangdong Province, China.E-mail:

Objective: To analyze the clinical features and prognosis of 28 children with myelodysplastic syndrome (MDS) and to screen the high risk factors affecting the prognosis so as to provide the new ideas for standard of clinical diagnosis and therapy.

Methods: The clinical data of 28 children with newly diagnosed MDS treated in our hospital from March 1994 to July 2016 were analyzed retrospectively, the features of disease onset and the results of laboratory examination were summarized, all MDS children were followed up, the prognosis and the high risk factors affecting the prognosis were evaluated.

Results: In all 28 MDS children, the ratio of male to female was 1. Read More

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December 2018

Aberrant histone modifications induced by mutant ASXL1 in myeloid neoplasms.

Int J Hematol 2018 Dec 5. Epub 2018 Dec 5.

Division of Cellular Therapy, Advanced Clinical Research Center, Division of Stem Cell Signaling, Center for Stem Cell Biology and Regenerative Medicine, Institute of Medical Science, The University of Tokyo, 4-6-1 Shirokanedai, Minato-ku, Tokyo, 1088639, Japan.

An epigenetic modulator Additional sex combs-like 1 (ASXL1) is recurrently mutated in myeloid neoplasms such as myelodysplastic syndromes (MDS), acute myeloid leukemia (AML) and myeloproliferative neoplasms (MPNs). ASXL1 mutations are also frequently detected in clonal hematopoiesis with indeterminate potential (CHIP), which is the clonal expansion of premalignant hematopoietic cells without any evidence of hematological malignancies. Thus, understanding the roles of ASXL1 in hematopoiesis and myeloid neoplasms is a clinically crucial issue. Read More

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December 2018

Myelodysplastic syndrome progression to acute myeloid leukemia at the stem cell level.

Nat Med 2018 Dec 3. Epub 2018 Dec 3.

Department of Cell Biology, Albert Einstein College of Medicine, Bronx, NY, USA.

Myelodysplastic syndromes (MDS) frequently progress to acute myeloid leukemia (AML); however, the cells leading to malignant transformation have not been directly elucidated. As progression of MDS to AML in humans provides a biological system to determine the cellular origins and mechanisms of neoplastic transformation, we studied highly fractionated stem cell populations in longitudinal samples of patients with MDS who progressed to AML. Targeted deep sequencing combined with single-cell sequencing of sorted cell populations revealed that stem cells at the MDS stage, including immunophenotypically and functionally defined pre-MDS stem cells (pre-MDS-SC), had a significantly higher subclonal complexity compared to blast cells and contained a large number of aging-related variants. Read More

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December 2018

Dichotomization of the new revised international prognostic scoring system for a better clinical stratification of patients with myelodysplastic syndromes.

Leuk Lymphoma 2018 Nov 30:1-6. Epub 2018 Nov 30.

a Department of Hematology , University Hospital Vall d'Hebron, University Autònoma of Barcelona (UAB) , Spain.

In clinical practice, patients with myelodysplastic syndromes (MDS) are usually classified in low or high-risk groups to take therapeutic decisions, conservative for low-risk, whereas active for high-risk. Nevertheless, in the Revised International Prognostic Scoring System (IPSS-R) is not well stated which patients are low or high-risk. This study was aimed to ascertain in 364 MDS patients which IPSS-R threshold better dichotomized in low vs. Read More

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November 2018
2 Reads

Pseudogout: An Autoimmune Paraneoplastic Manifestation of Myelodysplastic Syndrome.

Cureus 2018 Sep 27;10(9):e3372. Epub 2018 Sep 27.

Internal Medicine, University at Buffalo / Sisters of Charity Hospital, Buffalo, USA.

Myelodysplastic syndrome (MDS) is often associated with autoimmune paraneoplastic manifestations. Seronegative arthritis is among one of them. Very rarely, pseudogout demonstrated as paraneoplastic autoimmune manifestations of MDS has been adumbrated so far. Read More

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September 2018
2 Reads

An isolated der(1;21)(q10;q10) translocation in a patient with myelodysplastic syndrome: a case report.

Am J Blood Res 2018 5;8(3):17-20. Epub 2018 Oct 5.

Osaka General Hospital of West Japan Railway Company 1-2-22 Matsuzaki-cho, Abeno-ku, Osaka 545-0053, Japan.

Whole-arm translocations are relatively rare among hematological malignancies. There are a few reports on myeloid malignancies harboring der(1;21)(q10;q10). A 65-year-old male was referred to our hospital due to squamous cell carcinoma of the lung. Read More

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October 2018

Up-regulation of SPINT2/HAI-2 by Azacytidine in bone marrow mesenchymal stromal cells affects leukemic stem cell survival and adhesion.

J Cell Mol Med 2018 Nov 28. Epub 2018 Nov 28.

Hematology and Transfusion Medicine Center-University of Campinas/Hemocentro-UNICAMP, Campinas, São Paulo, Brazil.

The role of tumour microenvironment in neoplasm initiation and malignant evolution has been increasingly recognized. However, the bone marrow mesenchymal stromal cell (BMMSC) contribution to disease progression remains poorly explored. We previously reported that the expression of serine protease inhibitor kunitz-type2 (SPINT2/HAI-2), an inhibitor of hepatocyte growth factor (HGF) activation, is significantly lower in BMMSC from myelodysplastic syndromes (MDS) patients compared to healthy donors (HD). Read More

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November 2018

Retrospective evaluation of bone marrow cell morphology in a cohort of patients with isolated idic(20q-) karyotypic abnormalities.

Ann Hematol 2018 Nov 27. Epub 2018 Nov 27.

The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, 188 Shizi Street, Suzhou, 215006, Jiangsu, People's Republic of China.

Isochromosome 20q- (i(20q-)), as a rare reproducible chromosomal anomaly formed on the basis of 20q-, has not been commonly reported. Due to the rarity of this karyotypic anomaly, the bone marrow morphological characteristics of the patients with i(20q-) have not been clarified until now. In this study, the bone marrow cell morphology from MDS patients with isolated i(20q-), isolated 20q-, and normal karyotype was retrospectively compared and statistically analyzed. Read More

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November 2018

Cutaneous Leukemic Infiltrates Successfully Treated With Biomodulatory Therapy in a Rare Case of Therapy-Related High Risk MDS/AML.

Front Pharmacol 2018 13;9:1279. Epub 2018 Nov 13.

Department of Internal Medicine III, University Hospital Regensburg, Regensburg, Germany.

Cutaneous manifestations in hematologic malignancies, especially in leukemia, are not common and may be very variable. Here we report a very unusual case of a patient (female, 70 years old) who was admitted to the hospital in 2016 because of skin lesions on the face, the trunk of the body and the extremities. She had a history of breast cancer in the year 2004 (pT1b, pN0, cM0, L0, V0, R0) which had been resected and treated with adjuvant radiation and chemotherapy (cyclophosphamide, methotrexate, 5-fluorouracile) as well as psoriasis treated with methotrexate and cyclosporine. Read More

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November 2018
1 Read

[Advances in the treatment of juvenile myelomonocytic leukemia].

Zhongguo Dang Dai Er Ke Za Zhi 2018 Nov;20(11):958-963

Department of Pediatrics, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, Tianjin 300020, China.

Juvenile myelomonocytic leukemia (JMML) is a rare chronic myeloid leukemia in children and has the features of both myelodysplastic syndrome and myeloproliferative neoplasm. It is highly malignant and has a poor treatment outcome. Children with JMML have a poor response to conventional chemotherapy. Read More

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November 2018
2 Reads

Comparison of blast percentage calculated based on bone marrow all nucleated cells and non-erythroid cells in myelodysplastic syndromes with erythroid hyperplasia.

Ann Hematol 2018 Nov 24. Epub 2018 Nov 24.

Division of Hematology, Department of Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-shi, Tochigi-ken, 329-0498, Japan.

It is controversial whether blast percentage based on all nucleated cells (ANC) or non-erythroid cells (NEC) more accurately reflects the prognosis of patients with myelodysplastic syndromes (MDS). We considered that the impact of blast percentage on survival should be similar in MDS with erythroid hyperplasia (MDS-E) and MDS with no erythroid hyperplasia (MDS-NE), and from this perspective, we retrospectively analyzed 322 patients, including 44 with MDS-E and 278 with MDS-NE. Overall survival was similar between the MDS-E and MDS-NE groups (P = 0. Read More

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November 2018
1 Read

The prognostic impact of loss of chromosome 7 material detected by fluorescence in situ hybridization (FISH) in myeloid malignancies.

J Egypt Natl Canc Inst 2018 Dec 22;30(4):133-138. Epub 2018 Nov 22.

Clinical Pathology Department, Faculty of Medicine, Alexandria University, Alexandria, Egypt.

Background: Monosomy 7 (-7) or deletion in its long arm [del(7q)] is among the most common chromosomal abnormalities in myeloid malignancies. There are prognostic variations between -7 and del(7q) in acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS).

Aim: To describe the clinical characteristics, response to treatment, and survival of patients with primary AML and MDS having -7 or del(7q) detected by fluorescence in situ hybridization (FISH). Read More

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December 2018
2 Reads

Choosing a Reduced Intensity Conditioning Regimen for Allogeneic Stem Cell Transplantation, Fludarabine Busulfan versus Fludarabine Melphalan: A Systematic Review and Meta-analysis.

Biol Blood Marrow Transplant 2018 Nov 21. Epub 2018 Nov 21.

Mayo Clinic, Phoenix, AZ.

Fludarabine with busulfan (FB) or melphalan (FM) are two more commonly used reduced intensity conditioning regimens for allogeneic stem cell transplantation (HCT).We present a systematic review and meta-analysis of studies comparing these two RIC regimens. We searched electronic databases from inception through November 1, 2017 for literature search to identify relevant studies. Read More

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November 2018
3 Reads

A case of positive myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis.

Turk J Haematol 2018 Nov 23. Epub 2018 Nov 23.

Department of Pathology and Laboratory Medicine, Western University, London, Ontario, Canada.

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November 2018

Current and Future Treatment Options for Myelodysplastic Syndromes: More Than Hypomethylating Agents and Lenalidomide?

Drugs 2018 Nov 22. Epub 2018 Nov 22.

Medical Clinic and Policlinic I, Hematology and Cellular Therapy, Leipzig University Hospital, 04103, Leipzig, Germany.

Myelodysplastic syndromes are a heterogeneous group of bone marrow disorders that result in cytopenias and a propensity to develop secondary leukemia. While allogeneic transplantation still remains the only potential curative treatment option, it can only be offered to a limited number of patients. For the majority, who are not transplant candidates, treatment strategies cover iron chelation, growth factors, lenalidomide, and hypomethylating agents to improve cytopenia and potentially delay disease progression. Read More

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November 2018
2 Reads

Patterns of mutations in TP53 mutated AML.

Authors:
John S Welch

Best Pract Res Clin Haematol 2018 Dec 20;31(4):379-383. Epub 2018 Sep 20.

Department of Internal Medicine, Washington University, 660 Euclid Ave, Box 8007, St. Louis, MO 63110, USA. Electronic address:

TP53 mutated acute myeloid leukemia (AML) responds poorly to chemotherapy and has a short overall survival rate with a median of 5-9 months. Poor outcomes in TP53 mutated AML following chemotherapy have been observed and treatment options remain limited, although the presence of TP53 mutations alone should not be a barrier to therapy. Decitabine is emerging as an alternative treatment option for patients with TP53 mutated AML, although the agent has not been associated with deep molecular remissions and requires additional consolidation. Read More

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December 2018
1 Read

The role of hypomethylating agents prior to hematopoietic cell transplantation in myelodysplastic syndromes.

Best Pract Res Clin Haematol 2018 Dec 20;31(4):346-350. Epub 2018 Sep 20.

Hematology and Medical Oncology - Leukemia Program, Cleveland Clinic, 9500 Euclid Avenue, Desk CA-60, Cleveland, OH 44195, USA. Electronic address:

Therapies for myelodysplastic syndromes (MDS) are chosen depending on whether a patient has lower- or higher-risk disease. Hypomethylating agents are recommended as up-front therapy for all higher-risk patients, and lower-risk patients with multiple cytopenias. This article reviews the rationale for hypomethylating agents, their use or intensive chemotherapy pre-transplant, and data supporting pre-transplant MDS treatment. Read More

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December 2018

What biologic factors predict for transformation to AML?

Authors:
Rafael Bejar

Best Pract Res Clin Haematol 2018 Dec 23;31(4):341-345. Epub 2018 Oct 23.

UC San Diego Moores Cancer Center, 3855 Health Sciences Drive, San Diego, CA, 92093, USA. Electronic address:

Transformation of myelodysplastic syndromes (MDS) into secondary acute myeloid leukemia (sAML) is defined by an arbitrary boundary of ≥20% bone marrow blasts but does not necessarily reflect a defined biological transition. The more obvious distinction lies between MDS patients that have an isolated bone marrow failure phenotype and those with excess blasts. Subtyping of MDS might be more accurately stratified into clonal cytopenias and oligoblastic leukemias, using the degree of dysplasia and blast percentage as risk features, respectively, rather than as diagnostic criteria. Read More

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December 2018
4 Reads

DNA Methyltransferase Inhibitors and Their Therapeutic Potential.

Curr Top Med Chem 2018 Nov 20. Epub 2018 Nov 20.

Jiangsu Key Laboratory of Neuropsychiatric Diseases and Department of Medicinal Chemistry, College of Pharmaceutical Sciences, Soochow University, 199 Ren-Ai Road, Suzhou, Jiangsu 215123. China.

Aberrant DNA methylation at the 5-position of cytosine, catalyzed by DNA methyltransferases (DNMTs), is associated with not only various cancers by silencing of tumor suppressor genes but also other diseases. The DNMTs, especially the DNMT1, DNMT3A and DNMT3B, are often overexpressed in various cancer tissues and cell lines. DNMTs are important epigenetic targets for drug development since the DNA methylation is reversible. Read More

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November 2018
10 Reads

Paroxysmal nocturnal hemoglobinuria testing in patients with myelodysplastic syndrome in clinical practice-frequency and indications.

Curr Oncol 2018 Oct 31;25(5):e391-e397. Epub 2018 Oct 31.

Division of Hematology, St. Paul's Hospital and the University of British Columbia, Vancouver, BC.

Background: Myelodysplastic syndrome (mds) is characterized by peripheral blood cytopenias, with most patients developing significant anemia and dependence on red blood cell (rbc) transfusion. In paroxysmal nocturnal hemoglobinuria (pnh), mutations in the gene lead to lack of cell-surface glycosylphosphatidylinositol, allowing complement-mediated lysis to occur. Paroxysmal nocturnal hemoglobinuria results in direct antiglobulin test-negative hemolysis and cytopenias, and up to 50% of patients with mds test positive for pnh cells. Read More

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October 2018
2 Reads

Genome-Scale Single Nucleotide Resolution Analysis of DNA Methylation in Human Autosomal Dominant Polycystic Kidney Disease.

Am J Nephrol 2018 Nov 21;48(6):415-424. Epub 2018 Nov 21.

Department of Pathology, Dunedin School of Medicine, University of Otago, Dunedin, New

Background: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the formation of fluid-filled cysts in the kidney and end stage renal disease by the fourth or fifth decade of life. Mutations in the PKD1 gene account for 85% of all cases of ADPKD. No curative therapy exists for patients affected by this disease and an underexplored avenue for the treatment of ADPKD is the targeting of epigenetic changes that occur during cystogenesis. Read More

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November 2018
2 Reads

Targeting age-related inflammation in myelodysplastic syndromes.

Authors:
Yang Mei Peng Ji

Oncotarget 2018 Oct 23;9(83):35376-35377. Epub 2018 Oct 23.

Yang Mei: Department of Pathology, Feinberg School of Medicine, Northwestern University, Chicago, IL USA.

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October 2018
1 Read

Gastrointestinal Behcet's-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review.

Leuk Lymphoma 2018 Nov 20:1-7. Epub 2018 Nov 20.

a Department of Internal Medicine Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Assistance Publique-Hôpitaux de Paris , Hôpital Saint-Antoine , Paris , France.

We report the 11 cases of +8-MDS/MPN associated with Behcet's-like syndrome and compare them with Behcet's disease and Crohn's disease, pool with literature cases for analysis. Data for patients with +8-MDS/MPN and Behçet's-like syndrome were collected from MINHEMON. Eleven patients had Behcet's-like syndrome and +8-MDS/MPN (median age 75 years [IQR 65-87]; M/F ratio 0. Read More

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November 2018
5 Reads

TET2 rs2454206, TET2 rs12498609 and ASXL1 rs3746609 single nucleotide polymorphisms in patients with myelodysplastic syndromes.

Blood Cells Mol Dis 2019 Feb 7;74:44-50. Epub 2018 Nov 7.

Department of Hematology, The Second Hospital of Shanxi Medical University,382 Wuyi St, Taiyuan, Shanxi, China. Electronic address:

To study the association between TET2rs2454206, TET2rs12498609 and ASXL1rs3746609 and Myelodysplastic syndromes (MDS), a total of 90 MDS patients and 143 healthy volunteers were included. The clinical data, bone marrow samples of patients and peripheral blood samples of volunteers were obtained. We found TET2rs2454206 G/A genotype, TET2rs12498609 G/C genotype and ASXL1rs3746609 A/G genotype in 13. Read More

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February 2019

Activating p53 and Inhibiting Superenhancers to Cure Leukemia.

Trends Pharmacol Sci 2018 Dec;39(12):1002-1004

Leukemia Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA; Human Oncology and Pathogenesis Program, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY 10065, USA. Electronic address:

In a recent study, Minzel and colleagues identified a novel series of molecules that inhibit casein kinase 1α (CK1α), CDK7, and CDK9, resulting in p53 activation and preferential inhibition of superenhancer (SE)-driven transcription. This study demonstrates a highly effective therapeutic strategy combining p53 activation with suppression of SEs to promote the cooperative killing of leukemic cells. Read More

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December 2018
3 Reads

Patient-reported outcomes from a randomized phase II study of the deferasirox film-coated tablet in patients with transfusion-dependent anemias.

Health Qual Life Outcomes 2018 Nov 19;16(1):216. Epub 2018 Nov 19.

University College London, London, UK.

Background: Adherence to long-term chelation therapy in transfusion-dependent patients is critical to prevent iron overload-related complications. Once-daily deferasirox dispersible tablets (DT) have proven long-term efficacy and safety in patients ≥2 years old with chronic transfusional iron overload. However, barriers to optimal adherence remain, including palatability, preparation time, and requirements for fasting state. Read More

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November 2018
1 Read

Knock-in of the R394W mutation causes MDS and cooperates with to drive aggressive myeloid neoplasms in mice.

Oncotarget 2018 Oct 19;9(82):35313-35326. Epub 2018 Oct 19.

The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Wilms tumor 1 (WT1) is a zinc finger transcriptional regulator, and has been implicated as both a tumor suppressor and oncogene in various malignancies. Mutations in the DNA-binding domain of the gene are described in 10-15% of normal-karyotype AML (NK-AML) in pediatric and adult patients. Similar mutations have been reported in adult patients with myelodysplastic syndrome (MDS). Read More

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October 2018
1 Read
6.359 Impact Factor

Hepatocellular Carcinoma Arising in a Non-cirrhotic Liver with Secondary Hemochromatosis.

Intern Med 2018 Nov 19. Epub 2018 Nov 19.

Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Japan.

A 70-year-old man was admitted for treatment of a single liver nodule that was detected by contrast-enhanced computed tomography. Twenty years earlier, the patient had been diagnosed with myelodysplastic syndrome-refractory anemia and secondary hemochromatosis but had not received erythrocyte transfusions. The current histological, computed tomography, and magnetic resonance imaging findings revealed hepatocellular carcinoma (HCC) and non-cirrhotic liver hemochromatosis. Read More

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November 2018
3 Reads

Infection control in patients with myelodysplastic syndromes who are candidates for active treatment: Expert panel consensus-based recommendations.

Blood Rev 2018 Oct 28. Epub 2018 Oct 28.

Scientific Direction, IRCCS-CROB, Referral Cancer Center of Basilicata, Rionero in Vulture, PZ, Italy.

The improvement in supportive care and the introduction of new therapeutic agents, including lenalidomide and hypomethylating agents, in myelodysplastic syndromes have improved patients' outcomes; however, at the same time, the frequency and epidemiology of infections have changed. Therefore, the great strides in the indications and use of new treatment strategies for myelodysplastic syndromes need a parallel progress in the best approach to prophylaxis and supportive therapy for infections. Based on the recognition that the above issues represent an unmet clinical need in myelodysplastic syndromes, an Italian expert panel performed a review of the literature and composed a framework of the best recommendations for optimal infection control in patient candidates to receive active treatment for myelodysplastic syndromes. Read More

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October 2018
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Surveillance of Myelodysplastic Syndrome via Migration Analyses of Blood Neutrophils: A Potential Prognostic Tool.

J Immunol 2018 Nov 16. Epub 2018 Nov 16.

Institute for Experimental Immunology and Imaging, University Hospital, University Duisburg-Essen, 45147 Essen, Germany;

Autonomous migration is a central characteristic of immune cells, and changes in this function have been correlated to the progression and severity of diseases. Hence, the identification of pathologically altered leukocyte migration patterns might be a promising approach for disease surveillance and prognostic scoring. However, because of the lack of standardized and robust assays, migration patterns have not been clinically exploited so far. Read More

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November 2018
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