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Hematology (Amsterdam, Netherlands)

Luca Frison, Annamaria Lombardi, Ilaria Caputo, Gianpietro Semenzato, Fabrizio Fabris, Fabrizio Vianello
OBJECTIVES: The relevance of detecting antibodies against anticardiolipin, β2-glycoprotein I (β2gpI) or lupus anticoagulant (LA), collectively called antiphospholipid autoantibodies (APA), in subjects with immune thrombocytopenia (ITP) is still a debated issue. In particular, whether APA profile may affect the clinical course of ITP is unknown. METHODS: In this study, we report our experience in a cohort of ITP patients with APA with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment...
October 25, 2018: Hematology (Amsterdam, Netherlands)
Deyna Cardosa, Alexandra Ridout, Surabhi Nanda, Jo Howard, Susan E Robinson, Eugene Oteng-Ntim
OBJECTIVES: Maternal sickle cell disease (SCD) and multiple gestations are well known separately as causes of high-risk pregnancies, however, there is sparse information available on maternal and perinatal outcome when both conditions occur together. This case series describes the outcomes of women with maternal SCD and twin pregnancy in the largest single-center case series to date. METHODS: Retrospective identification of all twin pregnancies in maternal SCD patients between 2006 and 2016 at Guy's and St...
October 21, 2018: Hematology (Amsterdam, Netherlands)
Astrid Muyldermans, Lisa Florin, Helena Devos, Barbara Cauwelier, Jan Emmerechts
OBJECTIVES: The diagnosis of myelodysplastic syndrome (MDS) is not always straightforward in the absence of objective markers such as ringed sideroblasts, an excess of blasts or clonal cytogenetic abnormalities. Moreover, the lack of specificity of morphological dysplasia makes the differentiation between MDS and other causes of peripheral cytopenia difficult. The WHO 2016 classification of MDS recognizes multiparameter flow cytometry (MFC) as an adjuvant tool for MDS diagnosis. An easily applicable MFC protocol based on CD34 and CD45 is proposed by Ogata et al...
October 18, 2018: Hematology (Amsterdam, Netherlands)
Panchalee Satpanich, Ponlapat Rojnuckarin
INTRODUCTION: Studies in Western countries show that VTE recurrent rates are lower in the presence of a transient provoking factor, older age, female sex and/or hormonal use, while thrombophilia (factor V Leiden or prothrombin mutation) has no predictive role. This study aimed to determine the incidence and risk factors of recurrent VTE in Asian patients. METHOD: This is a retrospective cohort study in adult VTE patients who were diagnosed during 2004-2014, had no active cancer and followed-up for at least 1 year...
October 18, 2018: Hematology (Amsterdam, Netherlands)
Houfang Sun, Sheng Wei, Lili Yang
OBJECTIVES: Large granular lymphocyte (LGL) leukemia is a rare type of lymphoproliferative disease caused by clonal antigenic stimulation of T cells and natural killer (NK) cells. METHODS: In this review, we focus on the current knowledge of the immunological dysfunctions associated with LGL leukemia and the associated disorders coexistent with this disease. Novel therapeutic options targeting known molecular mechanisms are also discussed. RESULTS AND DISCUSSION: The pathogenesis of LGL leukemia involves the accumulation of gene mutations, dysregulated signaling pathways and immunological dysfunction...
October 18, 2018: Hematology (Amsterdam, Netherlands)
Chen Liu, Man Han, Lei Zhao, Mengjie Zhu, Qinzhu Xu, Ying Song, Hui Wang
OBJECTIVE: This research aimed to explore the significance of low activity of ADAMTS-13 in acute myeloid leukemia (AML) after bone morrow transplantation (BMT), and to evaluate the disease progress and prognosis of the patients with low or normal activity of ADAMTS-13 after BMT. METHODS: 46 AML patients were included in our research. ADAMTS-13 activity was measured before BMT. Their medical indicators were recorded one month after BMT. All the patients were followed up and their disease progression was evaluated afterwards...
October 16, 2018: Hematology (Amsterdam, Netherlands)
Liqiong Yao, Bei Liu, Li Jiang, Lanxia Zhou, Xiaoju Liu
OBJECTIVES: To investigate the association of cytotoxic T lymphocyte-associated antigen 4 (CTLA4) with immune thrombocytopenia (ITP). METHODS: A case-control association analysis of 277 Chinese Han children was performed. The tagging variants rs11571315 and rs3087243 in the CTLA4 gene were detected using polymerase chain reaction-restriction fragment length polymorphism method. The expression quantitative trait loci (eQTL) analysis and quantitative real-time polymerase chain reaction were performed to determine the relationship of CTLA4 with ITP...
October 14, 2018: Hematology (Amsterdam, Netherlands)
Jiasheng Xu, Kexin Li, Weimin Zhou
OBJECTIVE: To investigate the association between the polymorphism of methylenetetrahydrofolate reductase (MTHFR) gene and the formation of lower extremities deep venous thrombosis, and to evaluate the etiology of deep venous thrombosis. METHODS: Polymorphisms of the 677th site C/T in MTHFR gene for 101 patients with lower extremities deep venous thrombosis (DVT group) and 120 healthy subjects (control group) were detected by polymerase chain reaction with sequence-specific primers...
October 10, 2018: Hematology (Amsterdam, Netherlands)
Gülşah Barğı, Meral Boşnak Güçlü, Ayhan Gülsan Türköz Sucak
OBJECTIVES: Fatigue is a common symptom in allogeneic-hematopoietic stem cell transplantation (allogeneic-HSCT) recipients. However, effects of severe fatigue on pulmonary functions, blood cells, dyspnea, muscle strength, exercise capacity, depression and quality of life (QOL) in allogeneic-HSCT recipients are still unknown. Therefore, to compare pulmonary functions, blood levels, dyspnea, muscle strength, exercise capacity, depression, and QOL between allogeneic-HSCT recipients according to fatigue severity and to determine predictors of severe fatigue were aimed in the current study...
September 27, 2018: Hematology (Amsterdam, Netherlands)
Amira Mohamed Foad Shehata, Amira I Aldesoky, Suzy F Gohar
OBJECTIVES: Although many studies have assessed numerous molecular and immunohistochemical prognostic markers for diffuse large Bcell lymphoma (DLBCL), there is always a need for simple widely available markers. This study was planned to illustrate the clinical significance of baseline plasma fibrinogen levels in DLBCL patients. METHODS: We prospectively investigated 76 DLBCL patients treated with rituximab plus cyclophosphamide, vincristine, doxorubicin and hostacortine between August 2015 and February 2018...
September 10, 2018: Hematology (Amsterdam, Netherlands)
Preeti Tripathi, Priyanka Mishra, Ravi Ranjan, Seema Tyagi, Tulika Seth, Renu Saxena
OBJECTIVE: Factor VII deficiency is the commonest of the rare bleeding disorders with limited knowledge on clinical profile. The objective of this study was to study the prevalence and clinico-hematological profile of factor VII-deficient patients. METHODS: It is a retrospective observational study of probable inherited factor VII deficiency covering 18 months. Their clinical profile, family history, investigation and treatment records were studied in detail. RESULTS: The study group comprised of total 12 factor VII deficiency cases with mean age of 17...
September 7, 2018: Hematology (Amsterdam, Netherlands)
Signe Sperling, Pernille J Vinholt, Ulrik Sprogøe, Mark H Yazer, Henrik Frederiksen, Christian Nielsen
OBJECTIVES: Reduced platelet (PLT) function during storage has been shown for buffy-coat-derived platelet concentrates (BCP) and apheresis platelet units (AP), while for whole blood (WB) it has not been well studied. The aim of this study was to investigate PLT function in these blood products throughout storage using a novel flow cytometric assay. METHODS: Flow cytometric measurement of agonist-induced platelet aggregation, CD62P expression and PAC-1 binding during storage in BCP, AP (1-9 days at 20°C) and WB (1-21 days at 2-6°C)...
August 31, 2018: Hematology (Amsterdam, Netherlands)
Felix Gaytan-Morales, Francisco Alejo-Gonzalez, Alfonso Reyes-Lopez, Miguel Palomo, Laura Rodriguez-Romo, Laura Villareal-Martínez, Adriana Sandoval-González, Aracely Lopez-Facundo, Isidoro Tejocote-Romero, Rocio Cárdenas-Cardos, Marco Aguilar-Ortiz, Farina Arreguin-Gonzalez, Eduardo Baños-Rodriguez, Deyanira Cortes-Alva, Andrea Ellis-Irigoyen, Gladys García-Becerra, Marcela Rodriguez-Campos, Pablo Gonzalez-Montalvo, Oscar Gonzalez-Ramella, Alberto Olaya-Vargas
OBJECTIVE: Mature B-cell non-Hodgkin lymphoma (B-NHL) comprises more than 50% of all non-Hodgkin lymphoma (NHL) in children and adolescents. An official report published by the Mexican National Center for the Control and Prevention of Cancer in the Pediatric and Adolescent Populations, reported a lymphoma OS of 71% (including all Hodgkin and NHL). The Mexican Association of Pediatric Oncology and Hematology conducted a retrospective study to analyze the clinical characteristics and outcomes of children with diagnosis of B-NHL in Mexico, in order to perceive the main areas of improvement in the health care...
August 27, 2018: Hematology (Amsterdam, Netherlands)
Rajib De, Atreyee Dutta, Tuphan Kanti Dolai, Kakoli Ghosh, Ajanta Halder
OBJECTIVES: Interleukin-2 (alias: IL-2, TCGF, Lymphokine), a type of interleukin, is also a potent signalling molecule in the signalling cascade of the immune-mediated activation of T Lymphocytes leading to the destruction of haematopoietic stem cell (HSC) which is the basis of acquired aplastic anaemia (AAA). The objective was to study the association of IL-2 in the bone marrow plasma (BMP) and peripheral blood plasma (PBP) in AAA patients. METHODS: A total of 52 BMP and PBP-paired samples (both from the same patients) was collected from the confirmed AAA patients and 10 healthy individuals...
August 23, 2018: Hematology (Amsterdam, Netherlands)
José Carlos Jaime-Pérez, Goel Treviño-Reyna, Patrizia Aguilar-Calderón, Olga G Cantú-Rodríguez, Luis Javier Marfil-Rivera, David Gómez-Almaguer
OBJECTIVES: To demonstrate the importance of regional efforts to register features and report frequency of hematology diseases in the context of incomplete national registries. METHODS: Frequencies and salient characteristics of hematologic diseases in Northeast Mexico were documented in a reference center at a tertiary care university hospital during the decade 2005-2015. Disease categories were grouped by age, sex and diagnosis. Age group distribution followed WHO guidelines in years as children (0-17), adults (18-64) and elders (+65)...
December 2018: Hematology (Amsterdam, Netherlands)
Junyue Fang, Li Lin, Ying Wang, Dijin Lin, Chunyue Liu, Qiaozi Sunlong, Xianghua Lin
OBJECTIVES: To investigate the immune status of children with very severe aplastic anemia (VSAA), and evaluate the frequencies of CD20+ B cells and Regulatory T cells (Tregs) as potential markers for evaluating the therapeutic efficacy and prognosis. METHODS: We systematically analyzed CD20+ B cells and Tregs using Flow Cytometry in 36 children with VSAA (14 newly diagnosed cases and 22 cases in remission after therapy with HDIVIG + r-ATG + CSA). RESULTS: In newly diagnosed VSAA patients, the percentage of CD20+ B cells was higher than that in healthy children (P < ...
December 2018: Hematology (Amsterdam, Netherlands)
Guray Saydam, Ibrahim Celalettin Haznedaroglu, Leylagul Kaynar, Akif S Yavuz, Ridvan Ali, Birol Guvenc, Olga M Akay, Zafer Baslar, Ugur Ozbek, Mehmet Sonmez, Demet Aydin, Mustafa Pehlivan, Bulent Undar, Simten Dagdas, Orhan Ayyildiz, Gülnur Akin, Ilkiz M Dag, Osman Ilhan
OBJECTIVES: This report presents final results (24 months of follow-up) from the first prospective, national study of frontline nilotinib in chronic myeloid leukemia (CML) patients in Turkey. METHODS: Patients with newly diagnosed Philadelphia chromosome-positive CML in chronic phase (CML-CP; N = 112) received nilotinib 300 mg twice daily. The primary endpoint, which was the cumulative rate of major molecular response (MMR; BCR-ABL1 ≤ 0.1% on the International Scale [BCR-ABL1IS ]) by 12 months, was previously reported (66...
December 2018: Hematology (Amsterdam, Netherlands)
L De Roeck, L Michaux, K Debackere, E Lierman, P Vandenberghe, T Devos
OBJECTIVES: CML, PV, ET and PMF are so called classical MPN with distinct clinical phenotypes. The discovery of the BCR-ABL1 translocation and mutations in driver genes JAK2, MPL and CALR has provided novel insights in their pathogenesis. While these mutations are thought to be mutually exclusive, rare cases of MPN with coexisting driver mutations have been reported. However, little is known about the clinical, biological and molecular characteristics of these patients and the interaction of the neoplastic clones...
December 2018: Hematology (Amsterdam, Netherlands)
Rungtip Putburee, Arunee Jetsrisuparb, Supan Fucharoen, Amporn Tripatara
BACKGROUND: Patients with thalassaemia who received regular transfusions had increased iron accumulation, leading to iron overload, which was associated with oxidative stress. Mitochondrial ferritin, encoded by the FTMT gene is an iron-storage protein in the mitochondria. The aim of this work was to investigate the expression levels of FTMT in the reticulocytes of patients with alpha-thalassaemia who were regularly transfused and rarely transfused compared with healthy controls and to evaluate the relationships of the levels of FTMT mRNA with malondialdehyde (MDA) and ferritin in these patients...
December 2018: Hematology (Amsterdam, Netherlands)
Niloofar Naderi, Fariba Ebrahimzadeh, Mohammad Jazebi, Ali Namvar, Mehrdad Hashemi, Azam Bolhassani
Objectives Development of neutralizing antibodies against factor VIII is the major complication in hemophilia care which makes replacement therapies ineffective. The reports showed that inflammatory cytokines play an important role in inhibitor production. In the present study, the relationship between inhibitor development and the polymorphisms of two cytokine genes was studied in severe hemophiliac patients from Iran. Methods In this case-control study, three polymorphisms of immune regulatory genes [TGF-β (rs1982037) and IL-2 (rs2069762, rs4833248)] were analyzed in 100 Iranian hemophilia A patients divided into 55 inhibitor positive and 45 inhibitor negative patients using Tetra primer ARMS PCR, and DNA sequencing...
December 2018: Hematology (Amsterdam, Netherlands)
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