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    1370 results match your criteria Clinical Hematology[Journal]

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    Identification of a de novo ANK1 mutation in a Chinese family with hereditary spherocytosis.
    Hematology 2017 Nov 3:1-5. Epub 2017 Nov 3.
    d Prenatal Diagnosis Center , The Affiliated Hospital of Qingdao University , Qingdao , People's Republic of China.
    Objectives: Hereditary spherocytosis (HS) is a genetic heterogeneous disorder characterized by sphere-shaped erythrocytes on peripheral blood smear with a few clinical manifestations. As an important red cell membrane protein, ankyrin 1 can interact with transmembrane proteins and the membrane skeleton and mutations in the ankyrin 1 (ANK1) genes affect about half of all patients with HS. The purpose of this study was to investigate a Chinese Han family with HS to find out the causative gene mutation and explore the genotype-phenotype correlation which can provide the basis for the pathogenesis and prenatal diagnosis for this disease. Read More

    Estrogen receptor beta expression and prognosis of diffuse large B cell lymphoma.
    Hematology 2017 Oct 15:1-7. Epub 2017 Oct 15.
    a Department of Medicine, Faculty of Medicine , Chulalongkorn University and King Chulalongkorn Memorial Hospital (KCMH), Thai Red Cross Society , Bangkok , Thailand.
    Objectives: Estrogen receptor beta (ERβ)-selective agonists inhibited B cell lymphoma growth in animal models. However, a recent study found that higher ERβ expression in tissue from diffuse large B cell lymphoma (DLBCL) patients indicated a poorer survival. This study aimed to determine the ERβ expression in DLBCL tissue using immunohistochemistry and correlate with clinical outcomes. Read More

    Recommendations on the use of ruxolitinib for the treatment of myelofibrosis.
    Hematology 2017 Oct 12:1-7. Epub 2017 Oct 12.
    e Department of Hemato-Oncolocy , Erasme Hospital , Brussels , Belgium.
    Objectives: Myelofibrosis (MF) is a severe disease, with decreased life expectancy and heavy symptom burden. Ruxolitinib is the only approved pharmacotherapy for the treatment of MF patients. In Belgium, ruxolitinib is only reimbursed for MF patients with splenomegaly for whom the disease is categorized as intermediate-2 or high risk. Read More

    The potential association of tumor necrosis factor-βeta (252 G/A) cytokine gene polymorphism with immune thrombocytopenic purpura among Egyptian children.
    Hematology 2017 Oct 12:1-5. Epub 2017 Oct 12.
    e Faculty of Medicine , Beni-Suef University , Beni-Suef , Egypt.
    Objectives: The main objective of this study was to study tumor necrosis factor beta (TNFB) + 252G/A gene polymorphism, known to be related to autoimmunity, in immune thrombocytopenia (ITP) patients. We also aimed to investigate the association between TNFB + 252G/A polymorphism and susceptibility to develop persistent/chronic ITP.

    Methods: One hundred pediatric ITP patients, as well as 50 age- and sex-matched healthy Egyptian subjects, were included. Read More

    Tolerability and safety of the intravenous immunoglobulin octagam(®) 10% in patients with immune thrombocytopenia: a post-authorisation safety analysis of two non-interventional phase IV trials.
    Hematology 2017 Oct 11:1-6. Epub 2017 Oct 11.
    c Octapharma Inc. , Hoboken , NJ , USA.
    Objectives: To provide detailed data on the tolerability and safety of octagam(®) 10%, a ready-to-use intravenous immunoglobulin, in a subgroup of patients with immune thrombocytopenia (ITP) involved in an integrated analysis of post-authorisation safety surveillance (PASS) studies.

    Methods: A subgroup analysis was conducted using data collected from two non-interventional studies that included patients with ITP treated with octagam(®) 10%. Patients were observed and monitored for possible adverse drug reactions (ADRs) during or after administration of octagam(®) 10%, with a particular focus on thromboembolic events (TEEs). Read More

    EgoNet identifies differential ego-modules and pathways related to prednisolone resistance in childhood acute lymphoblastic leukemia.
    Hematology 2017 Oct 11:1-7. Epub 2017 Oct 11.
    a Department of Pediatrics , The Affiliated Hospital of Qingdao University , Qingdao , Shandong , People's Republic of China.
    Purpose: To extract feature ego-modules and pathways in childhood acute lymphoblastic leukemia (ALL) resistant to prednisolone treatment, and further to explore the mechanisms behind prednisolone resistance.

    Materials And Methods: EgoNet algorithm was used to identify candidate ego-network modules, mainly via constructing differential co-expression network (DCN); selecting ego genes; collecting ego-network modules; refining candidate modules. Afterwards, statistical significance was calculated for these candidate modules. Read More

    CALR, JAK2 and MPL mutation status in Argentinean patients with BCR-ABL1- negative myeloproliferative neoplasms.
    Hematology 2017 Oct 9:1-4. Epub 2017 Oct 9.
    a Cátedra de Hematología, Facultad de Ciencias Bioquímicas y Farmacéuticas , Universidad Nacional de Rosario , Guaraní 3012, Rosario , Argentina.
    Objectives: To establish the frequency of JAK2, MPL and CALR mutations in Argentinean patients with BCR-ABL1-negative  myeloproliferative neoplasms (MPN) and to compare their clinical and haematological features.

    Methods: Mutations of JAK2V617F, JAK2 exon 12, MPL W515L/K and CALR were analysed in 439 Argentinean patients with BCR-ABL1-negative MPN, including 176 polycythemia vera (PV), 214 essential thrombocythemia (ET) and 49 primary myelofibrosis (PMF).

    Results: In 94. Read More

    Multivariate analysis of prognosis for patients with natural killer/T cell lymphoma-associated hemophagocytic lymphohistiocytosis.
    Hematology 2017 Oct 6:1-7. Epub 2017 Oct 6.
    a Department of Hematology , Beijing Friendship Hospital, Capital Medical University , Beijing , People's Republic of China.
    Objective: A major cause of hemophagocytic lymphohistiocytosis (HLH) is malignant neoplasms of the blood system, among which NK/T cell lymphoma is one of the most common risk factors. Patients with NK/T cell lymphoma hemophagocytic lymphohistiocytosis (NK/T-LAHS) have a worse prognosis and higher mortality. We aimed to explore the factors that affect the prognosis of NK/T-LAHS. Read More

    FC gamma receptor polymorphisms in patients with immune thrombocytopenia.
    Hematology 2017 Sep 23:1-6. Epub 2017 Sep 23.
    a University Clinic for Hematology , Faculty of Medicine , Skopje , Republic of Macedonia.
    Introduction: Immune thrombocytopenia (ITP) is an autoimmune blood disease of unknown etiology. The aim of our study was to investigate a possible role of FCGR2A and FCGR3A polymorphisms in the development of primary ITP.

    Methods: We analyzed 125 adult patients with ITP and 120 healthy controls. Read More

    Flow cytometric analysis of patients with hereditary spherocytosis - an Indian scenario.
    Hematology 2017 Sep 15:1-6. Epub 2017 Sep 15.
    b Department of Hematology , All India Institute of Medical Sciences (AIIMS) , New Delhi , India.
    Objectives: Flow cytometry osmotic fragility test (FC-OFT) was a recently introduced screening test for hereditary spherocytosis (HS). This study was conducted to evaluate the utility of FC-OFT in all newly diagnosed cases of HS, to compare its diagnostic value with conventional OFT and to correlate with clinical disease severity.

    Methods: In this study, the percentage of residual red cells (%RRC) was measured using flow cytometer after creating a red cell suspension. Read More

    High absolute basophil count is a powerful independent predictor of inferior overall survival in patients with primary myelofibrosis.
    Hematology 2017 Sep 14:1-7. Epub 2017 Sep 14.
    a Hematology Department , University Hospital Dubrava , Zagreb , Croatia.
    Objectives: To investigate the clinical and prognostic significance of absolute basophil count (ABC) in patients with primary myelofibrosis (PMF).

    Methods: We retrospectively investigated 58 patients with PMF treated in our institution in the period from 2006 to 2017. ABC was obtained in addition to other hematological and clinical parameters. Read More

    The impact of FLT3 mutations on treatment response and survival in Chinese de novo AML patients.
    Hematology 2017 Sep 6:1-8. Epub 2017 Sep 6.
    f Institute of Medical Biotechnology , Suzhou Vocational Health College , Suzhou , China.
    Objective: Two distinct forms of FMS-like tyrosine kinase 3 (FLT3) mutations, internal tandem duplication (ITD) in the juxtamembrane domain and point mutation within the activation loop of the tyrosine kinase domain (TKD), have been identified in considerable number of patients with AML. This study was aimed to analyze the impacts of these mutations on clinical outcomes, and assess the efficacy of different therapeutic regimens (allo-HSCT, sorafenib, or conventional chemotherapy) for AML patients with FLT3 mutations after the standard induction therapy.

    Materials And Methods: We analyzed DNA samples from 158 consecutive de novo AML patients (18-60 years, excluding APL) with FLT3 mutations between July 2010 and October 2015. Read More

    The impact of Fc gamma receptor IIa and IIIa gene polymorphisms on the therapeutic response of rituximab in Egyptian adult immune thrombocytopenic purpura.
    Hematology 2017 Aug 31:1-6. Epub 2017 Aug 31.
    a Clinical Hematology Unit - Internal Medicine Department, Faculty of Medicine , Cairo University , Cairo , Egypt.
    Background: In chronic immune thrombocytopenic purpura (ITP), rituximab removes the harmful autoantibodies through antibody-dependent cellular cytotoxicity. The response to rituximab in ITP is variable; the effectiveness of rituximab is influenced by the process of activation of effector fragment C gamma receptors (FcγRs). Genetic factors may affect the response to rituximab. Read More

    Can biomarkers of coagulation, platelet activation, and inflammation predict mortality in patients with hematological malignancies?
    Hematology 2017 Aug 20:1-7. Epub 2017 Aug 20.
    b Department of Chemical Pathology, Faculty of Medicine , Cairo University , Cairo , Egypt.
    Background: Patients with cancer commonly demonstrate laboratory evidence for hypercoagulability. Coagulation and inflammation play a role in the pathophysiology of hematological malignancies and the correlation between hypercoagulability and inflammation with tumor outcomes and the patient's prognosis are well studied.

    Objective: To identify an association between hemostasis activation, fibrinolysis and inflammation with mortality in patients with hematological malignancies to determine their prognostic significance. Read More

    Human platelet antigen 1, 2 and 5 gene polymorphisms in Egyptians and their potential association with susceptibility to immune thrombocytopenic purpura in Egyptian patients.
    Hematology 2017 Aug 20:1-6. Epub 2017 Aug 20.
    a Clinical and Chemical Pathology, Faculty of Medicine , Cairo University , Cairo , Egypt.
    Objectives: This study determined the incidence of HPA1, HPA2 and HPA5 polymorphisms in 120 Egyptian immune thrombocytopenic purpura (ITP) patients and 120 healthy Egyptian subjects.

    Methods: Human platelet antigen (HPA) genotyping was done using the polymerase chain reaction-restriction fragment length polymorphism.

    Results: The frequency of HPA1 allele a and b was 78. Read More

    Double vs. single cord blood transplantation in adolescent and adult hematological malignancies with heavier body weight (≥50 kg).
    Hematology 2017 Aug 10:1-9. Epub 2017 Aug 10.
    a Department of Hematology , Anhui Provincial Hospital, Anhui Medical University , Hefei , China.
    Background: Double-unit cord blood transplantation (CBT) can be used to overcome the limitation of single-unit CBT with low cell content for adults and larger adolescents. However, whether double-unit CBT is superior to single-unit CBT remains controversial.

    Methods: We reviewed the medical records of 228 consecutive hematological malignancies who received CBT between November 2005 and December 2013. Read More

    Immunophenotypic analysis of adult patients with T-cell lymphoblastic lymphoma treated with hyper-CVAD.
    Hematology 2017 Aug 4:1-6. Epub 2017 Aug 4.
    a Department of Hematology and Cell Therapy , Aichi Cancer Center Hospital , Nagoya , Japan.
    Objectives: Immunophenotype is an important prognostic factor for childhood and adult T-cell acute lymphoblastic leukemia. However, immunophenotypic data from adult patients with T-cell lymphoblastic lymphoma (T-LBL) are scarcely available.

    Methods: Subjects were unselected adult patients with T-LBL who were treated with intensive chemotherapy. Read More

    Interaction between Hb E and Hb Yala (HBB:c.129delT); a novel frameshift beta globin gene mutation, resulting in Hemoglobin E/β(0) thalassemia.
    Hematology 2017 Aug 3:1-5. Epub 2017 Aug 3.
    b Siriraj Integrated Center of Excellence for Thalassemia (SiiCOE-T) , Faculty of Medicine Siriraj Hospital, Mahidol University , Bangkok , Thailand.
    Objectives: There are more than 200 known mutations found in patients with β-thalassemia, a possibility to identify an unknown or novel mutation becomes less possible. Here, we report a novel mutation in a patient from Thailand who presented with chronic hemolytic anemia.

    Methods: A comprehensive hematology and DNA analysis was applied in the index patient and her mother. Read More

    Epidemiologic study of major complications in adolescent and adult patients with thalassemia in Northeastern Thailand: the E-SAAN study phase I.
    Hematology 2017 Jul 31:1-6. Epub 2017 Jul 31.
    d Center for Research and Development of Medical Diagnostic Laboratories, Faculty of Associated Medical Science , Khon Kaen University , Khon Kaen , Thailand.
    Introduction: Thalassemia-related complications are one of the main factors that increase morbidity and mortality in aging patients with thalassemia. This study was aimed to report the prevalence and clinical risk factors for the complications in thalassemia.

    Methods: A multi-center prospective cohort study was conducted in patients with thalassemia aged ≥10 years old. Read More

    Microsatellite Instability and Promoter Hypermethylation of DNA repair genes in Hematologic Malignancies: a forthcoming direction toward diagnostics.
    Hematology 2017 Jul 20:1-6. Epub 2017 Jul 20.
    a Department of Integrative Biology , Vellore Institute of Technology , Vellore , Tamil Nadu , India.
    Objective: The objective of our review is to highlight the significance of microsatellite hypervariation in diagnostics of hematologic malignancies.

    Methods: For the past few decades, extensive experiments in cancer research have explored all the possible pathways and a number of deleterious mutations that either make the tumor suppressor genes (TSGs) dysfunctional or cause the proto-oncogenes to behave abnormally by changing the cellular phenotype hence rendering disease. To prevent the deleterious effects of mutations and to protect the genomic integrity, our system possesses multiple repair mechanisms. Read More

    Characteristics of bone marrow cell dysplasia and its effectiveness in diagnosing myelodysplastic syndrome.
    Hematology 2017 Jul 4:1-12. Epub 2017 Jul 4.
    a Department of Medical Laboratory , First Affiliated Hospital of Sun Yat-sen University , Guangzhou , China.
    Background: Although dysplasia plays an important role in the diagnosis of myelodysplasia syndrome (MDS), its morphologic variety and irregularity result in difficulties in its clinical application.

    Methods: Bone marrow smears from cases with MDS and non-clonal disease were collected and performed microscopy analysis. We respectively recorded the percentage of specific dysplastic cells (PSDC) and incidence of specific dysplasia (ISD) of each dysplastic type in three hematopoietic cell lineages for the comprehensive analysis of diagnostic efficacy to MDS. Read More

    Pretransplant serum FT3 levels in recipients predict early non-relapse mortality after myeloablative allogeneic haematopoietic cell transplantation from matched sibling donors.
    Hematology 2017 Jun 26:1-6. Epub 2017 Jun 26.
    b Jiangsu Institute of Hematology , The First Affiliated Hospital of Soochow University , Suzhou , People's Republic of China.
    Objectives: Although decreased thyroid function is negatively correlated with clinical outcomes in critically ill patients, its role in allogeneic haematopoietic cell transplantation (allo-HCT) has not been sufficiently described.

    Methods: The associations between pre-conditioning thyroid hormone concentrations and transplant-related complications in 474 adult patients with haematologic malignancies who underwent myeloablative allo-HCT were assessed.

    Results: A receiver-operating characteristic curve showed that the baseline serum-free triiodothyronine 3 (FT3) level had an excellent predictive value for non-relapse mortality (NRM) within 100 days in sibling HCT with an area under the curve of 0. Read More

    Neurocognitive dysfunction in children with β thalassemia major: psychometric, neurophysiologic and radiologic evaluation.
    Hematology 2017 Dec 16;22(10):617-622. Epub 2017 Jun 16.
    e Department of Pediatric, Pediatric Neurology Unit , Mansoura University , Mansoura , Egypt.
    Objective: To evaluate the impact of iron chelating drugs and serum ferritin on the neurocognitive functions of patients with β thalassemia major (β-TM), using psychometric, neurophysiologic and radiologic tests.

    Methods: Eighty children with β-TM were enrolled into the study and were compared to 40 healthy controls. All participants were evaluated by measuring serum ferritin, neurocognitive assessment by Benton Visual Retention Test, Wechsler Intelligence Scale for Children, Wisconsin Card Sort Test, P300 and magnetic resonance spectroscopy (MRS). Read More

    Revisiting beta thalassemia intermedia: past, present, and future prospects.
    Hematology 2017 Dec 7;22(10):607-616. Epub 2017 Jun 7.
    b Department of Internal Medicine , American University of Beirut Medical Center , Beirut , Lebanon.
    Background: The spectrum of thalassemias is wide ranging from thalassemia minor, which consists of mild hypochromic microcytic anemia without obvious clinical manifestations, to thalassemia major (TM), which is characterized by severe anemia since the first years of life and is transfusion dependent. Thalassemia intermedia (TI) describes those patients with mild or moderate anemia.

    Objective: To describe the genetic features and major clinical complications of TI, and the therapeutic approaches available in the management of this disease. Read More

    Relapse of childhood acute lymphoblastic leukemia and outcomes at a reference center in Latin America: organomegaly at diagnosis is a significant clinical predictor.
    Hematology 2017 Jun 3:1-9. Epub 2017 Jun 3.
    a Department of Hematology , Dr. José Eleuterio González University Hospital of the School of Medicine, Universidad Autónoma de Nuevo León , Monterrey , Mexico.
    Objective: Relapse is the major cause of treatment failure in acute lymphoblastic leukemia (ALL) of childhood; it is more frequent among high-risk patients from low-middle income than from high-income countries. The frequency, sites and outcome of relapsed ALL in children of northeast Mexico over a decade was documented.

    Methods: A retrospective analysis of 246 children belonging to a low-income group <16 years with de novo ALL during 2004-2015 was performed. Read More

    Safety and efficacy of Ofatumumab in chronic lymphocytic leukemia: a systematic review and meta-analysis.
    Hematology 2017 Dec 3;22(10):578-584. Epub 2017 Jun 3.
    a Medical Institution Conducting Clinical Trials for Human Used Drug, The Fifth People's Hospital of Shanghai , Fudan University , Shanghai , China.
    Objectives: Increasing numbers of clinical studies have been carried out to investigate the therapeutic effect of Ofatumumab for patients with chronic lymphocytic leukemia (CLL) but no studies have yet reported a pooled estimate of the treatment effect. We performed a meta-analysis of evidence from 13 clinical trials to assess effectiveness and safety of Ofatumumab-based therapy in patients with CLL.

    Methods: Relevant publications from PubMed, Web of Science, Embase, and ClinicalTrials. Read More

    Clinical characteristics, molecular profile and outcomes of myeloid sarcoma: a single institution experience over 13 years.
    Hematology 2017 Jun 2:1-8. Epub 2017 Jun 2.
    b Division of Hematology and Oncology , Winthrop P. Rockefeller Cancer Institute, University of Arkansas for Medical Sciences , Little Rock , AR , USA.
    Background: Myeloid sarcoma (MS) is characterized by extramedullary infiltration by immature myeloid cells. Owing to rarity of this disease, the clinical features and overall outcomes are yet to be clarified.

    Objective: To define clinical characteristics, epidemiology, pathologic findings, treatment options and outcomes in MS. Read More

    The role of bone marrow microenvironment in platelet production and their implications for the treatment of thrombocytopenic diseases.
    Hematology 2017 Dec 1;22(10):630-639. Epub 2017 Jun 1.
    a Department of Hematology, South Campus Ren Ji Hospital, School of Medicine , Shanghai Jiao Tong University , Shanghai , PR China.
    Objectives: Impaired platelet production has been found to be an important pathological mechanism of thrombocytopenia in many diseases. Platelet generation is a complex process that mainly occurs in the bone marrow, and thus is closely regulated by the bone marrow microenvironment. This review attempts to summarize the most current knowledge referring the role of bone marrow microenvironment in the regulation of platelet production. Read More

    Cardiovascular events during carfilzomib therapy for relapsed myeloma: practical management aspects from two case studies.
    Hematology 2017 Dec 25;22(10):585-591. Epub 2017 May 25.
    c Department of Clinical Development , Amgen Inc., One Amgen Center Drive , Thousand Oaks , CA , USA.
    Objectives and importance: Patients with multiple myeloma (MM) have an increased risk of cardiovascular comorbidities due to disease burden and treatment-related risk factors. Proteasome inhibitors, including bortezomib and carfilzomib, are effective and generally well tolerated anti-MM agents. However, cardiovascular-related toxicities have been reported with this class of agents, the mechanisms of which are not fully understood. Read More

    Kidney injury in infants and children with iron-deficiency anemia before and after iron treatment.
    Hematology 2017 Oct 16;22(9):565-570. Epub 2017 May 16.
    c Pediatric Hematology/Oncology Unit, Pediatrics Department, Faculty of Medicine , Mansoura University , Mansoura, Egypt.
    Background: Our study aimed to investigate the effects of iron-deficiency anemia (IDA) on renal tubular functions before and after iron treatment for infants and children with IDA. We measured urinary levels of two kidney injury markers: neutrophil gelatinase-associated lipocalin (NGAL) and liver-type fatty acid-binding protein (L-FABP).

    Material And Methods: Thirty-six infants and children with IDA and 20 matched healthy controls were included. Read More

    Routine abdominal ultrasonography has limited value in the care for patients with indolent systemic mastocytosis.
    Hematology 2017 Oct 10;22(9):544-547. Epub 2017 May 10.
    a Department of Internal Medicine, Section of Clinical Immunology , Erasmus University Medical Centre , Rotterdam , Netherlands.
    Objectives: Systemic mastocytosis (SM) is a myeloproliferative disease characterized by the accumulation of aberrant mast cells. Since advanced subtypes of SM can lead to organ dysfunction and shortened survival, timely recognition of progressive disease is important for the adequate treatment of SM patients.

    Methods: Here, we report the results of our cohort study on the value of routine abdominal ultrasonography for the detection of progression of indolent systemic mastocytosis (ISM). Read More

    Analysis of clinical characteristics and prognostic factors of multiple myeloma: a retrospective single-center study of 787 cases.
    Hematology 2017 Sep 2;22(8):472-476. Epub 2017 May 2.
    a Department of Hematology , The First Affiliated Hospital, Zhejiang University , Hangzhou , China.
    Objective: This study aims to explore the clinical features of multiple myeloma (MM) and the influence of various prognostic factors on survival.

    Methods: A retrospective analysis, consisting of clinical characteristics analysis and laboratory examinations, was performed on 787 MM patients. Clinical and laboratory parameters were analyzed by multivariate process and compared across different groups. Read More

    A child with severe iron-deficiency anemia and a complex TMPRSS6 genotype.
    Hematology 2017 Oct 27;22(9):559-564. Epub 2017 Apr 27.
    a Department of Human Pathology of Adult and Developmental Age 'Gaetano Barresi' , 'Gaetano Martino' University Hospital of Messina , Messina , Italy.
    Objectives: We report a case of a 7-year-old girl with severe hypochromic microcytic anemia, who was unresponsive to classical iron supplements. We suspected IRIDA, iron-refractory iron-deficiency anemia, a genetic iron metabolism disorder, caused by TMPRSS6 variations. TMPRSS6 encodes matriptase-2, a negative regulator of hepcidin, and its pathological variants are related to normal to high levels of hepcidin. Read More

    Acquired hemophilia A: a review of recent data and new therapeutic options.
    Hematology 2017 Oct 25;22(9):514-520. Epub 2017 Apr 25.
    a Italian National Blood Centre, National Institute of Health , Rome , Italy.
    Objectives: Acquired hemophilia A (AHA) is a rare, but potentially life-threatening, bleeding disorder caused by an autoantibody against factor VIII that interferes with its coagulant function.

    Methods: We performed a narrative review focusing on the diagnostic aspects of AHA and on the current treatment strategies with particular regard to new data and therapeutic developments.

    Results: The management of this severe hemorrhagic disorder is based on the control of bleeding with the use of bypassing agents and on the utilization of a variety of immunosuppressant agents with the goal of eliminating the autoantibody permanently. Read More

    Characteristics and management of primary and other immune thrombocytopenias: Spanish registry study.
    Hematology 2017 Sep 17;22(8):484-492. Epub 2017 Apr 17.
    a Hospital Universitario y Politécnico la Fe , Valencia , Spain.
    Background: The natural history and its modulation by treatments administered for immune thrombocytopenia (ITP) in the clinical practice remains unknown. In addition, little information is available on the characteristics and management of ITP in Spain.

    Methods: We conducted an observational, multicenter, registry in 70 Hematology Services from Spain between 2009 and 2011, which included children from 2 months of age and adults with primary ITP or another ITP diagnosed within the last 6 months (platelet count [PC] < 100 × 10(9)/l). Read More

    Prognostic value of the soluble interleukin-2 receptor level after patients with follicular lymphoma achieve a response to R-CHOP.
    Hematology 2017 Oct 17;22(9):521-526. Epub 2017 Apr 17.
    a Division of Hematology, Department of Medicine , Jichi Medical University , Tochigi , Japan.
    Objectives: Follicular lymphoma (FL) is a clinically and biologically heterogeneous disease. Therefore, it is important to identify factors that can predict its clinical outcome.

    Methods: We retrospectively evaluated the usefulness of soluble interleukin-2 receptor (sIL-2R) levels after R-CHOP (posttreatment sIL-2R) in 72 patients with newly diagnosed FL who had either a complete response (CR) or partial response. Read More

    Elevation of CD16(+)CD56(+) NK-cells and down-regulation of serum interleukin-21 (IL-21) and IL-1α after splenectomy in relapsed hemophagocytic lymphohistiocytosis of unknown cause.
    Hematology 2017 Sep 15;22(8):477-483. Epub 2017 Apr 15.
    a Department of Hematology, Beijing Friendship Hospital , Capital Medical University , Beijing , China.
    Objectives: Encouraging progress has been made in application of splenectomy in the treatment of relapsed hemophagocytic lymphohistiocytosis (HLH) of unknown cause. The aim was to determine the roles of lymphocyte subpopulations and inflammatory cytokines in splenectomy.

    Methods: We retrospectively analyzed changes in lymphocyte subpopulations and levels of inflammatory cytokines at different time-points before and after splenectomy in the patients with relapsed HLH of unknown cause, as well as the correlations between these changes and the disease prognosis. Read More

    Clinical and hematological relevance of JAK2 V617F and CALR mutations in BCR-ABL-negative ET patients.
    Hematology 2017 Dec 13;22(10):599-606. Epub 2017 Apr 13.
    a Human Genetics Laboratory, Department of Pathology, Faculty of Medicine, Ramathibodi Hospital , Mahidol University , Bangkok , Thailand.
    Background: Classical BCR-ABL1-negative myeloproliferative neoplasms (MPNs) including polycythemia vera, essential thrombocythemia (ET), and primary myelofibrosis frequently harbor JAK2, MPL, and CALR somatic mutations.

    Methods: AS-PCR for JAK2 V617F, pyrosequencing for MPL W515L/K, and PCR-fragment analysis for CALR exon 9 mutations were established to analyze genomic DNA isolated from peripheral blood samples of 58 newly diagnosed ET patients in Thailand.

    Results: JAK2 V617F was detected in 41 patients (71%) and CALR exon 9 mutation was positive in eight patients (14%), whereas no mutation of MPL W515L/K was observed in this study. Read More

    Value of speckle tracking echocardiography for detection of clinically silent left ventricular dysfunction in patients with β-thalassemia.
    Hematology 2017 Oct 12;22(9):554-558. Epub 2017 Apr 12.
    e CMR Department, Rajaei Cardiovascular Medical and Research Center , Iran University of Medical Sciences , Tehran , Iran.
    Objective: β-Thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring chronic transfusion therapy. Cardiac involvement is the main cause of death in patients with thalassemia major. The narrow border is between overt myocardial dysfunction and clinically silent left ventricular (LV) dysfunction in patients with thalassemia. Read More

    Clinical usefulness of FDG-PET/CT for the evaluation of various types of adult T-cell leukemia.
    Hematology 2017 Oct 11;22(9):536-543. Epub 2017 Apr 11.
    a Division of Endocrinology, Diabetes and Metabolism, Hematology and Rheumatology (Second Department of Internal Medicine), Graduate School of Medicine , University of the Ryukyus , Nishihara , Japan.
    Purpose: The aim was to explore undefined useful indices for clinically grading adult T-cell leukemia (ATL) using [(18)F] 2-fluoro-2-deoxyglucose (FDG) - positron emission tomography/computed tomography (PET/CT).

    Methods: A total of 28 patients with ATL (indolent, 9; aggressive, 19) were enrolled; all patients with aggressive ATL underwent FDG-PET/CT before chemotherapy. Patients with indolent ATL underwent FDG-PET/CT at the time of suspected disease progression and/or transformation; some received lymph node biopsy. Read More

    Predictive parameters for imatinib failure in patients with chronic myeloid leukemia.
    Hematology 2017 Sep 22;22(8):460-466. Epub 2017 Mar 22.
    a Clinic for Hematology , Clinical Center of Serbia , Belgrade , Serbia.
    Objective: Until recently, imatinib was the standard first-line treatment in chronic myeloid leukemia (CML). The inclusion of nilotinib and dasatinib as first-line options in CML raised a debate on treatment selection. The aim of our study was to analyze predictive parameters for imatinib response as the first-line treatment of CML patients. Read More

    The role of follicular T helper cells in patients with malignant lymphoid disease.
    Hematology 2017 Aug 10;22(7):412-418. Epub 2017 Mar 10.
    a Department of Hematology , The Second Affiliated Hospital of Soochow University , Suzhou , China.
    Objectives: To investigate the dynamic change of follicular T helper cells (TFH) in patients with malignant lymphoid disease (MLD) and to explore its clinical significance.

    Methods: The dynamic change of TFH cells, ICOS(+)- and PD-1(+) TFH cells at pretreatment and different treatment periods was determined by flow cytometry in 85 MLD patients. Concentration of interleukin 21 (IL-21) was evaluated by ELISA, and the correlation between clinical prognosis and the ratio of TFH cells was analyzed. Read More

    Upregulation of microRNA-21 is a poor prognostic marker in patients with childhood B cell acute lymphoblastic leukemia.
    Hematology 2017 Aug 2;22(7):392-397. Epub 2017 Mar 2.
    c Medical Oncology Department, Faculty of Medicine , Zagazig University , Zagazig , Egypt.
    Objectives: Many studies have demonstrated that microRNA-21 (miR-21) is an oncogene and is upregulated in tumor tissue. However, its association with B-cell acute lymphoblastic leukemia (B-ALL) remains poorly understood.

    Methods: The expression of miR-21 was detected by real-time quantitative PCR in 75 children with de novo B-ALL as well as in 50 healthy controls. Read More

    Protector effect of α-thalassaemia on cholecystitis and cholecystectomy in sickle cell disease.
    Hematology 2017 Aug 21;22(7):444-449. Epub 2017 Feb 21.
    c Department of Clinical Analysis and Toxicology, School of Pharmacy , Rio de Janeiro Federal University (UFRJ) , Rio de Janeiro , Brazil.
    Objectives: Cholecystitis is one of the complications of symptomatic cholelithiasis responsible for high levels of morbidity of sickle cell disease (SCD) patients. Here, we investigated the possible protective role of single gene deletions of α-thalassaemia in the occurrence of cholelithiasis and cholecystitis in SCD patients, as well as the cholecystectomy requirements.

    Methods: The α-globin genotype was determined in 83 SCD patients using the multiplex-polymerase chain reaction and compared with clinical events. Read More

    Molecular and clinical analysis of haemoglobin Lepore in Campania, a region of Southern Italy.
    Hematology 2017 Aug 20;22(7):437-443. Epub 2017 Feb 20.
    a UOSD Malattie rare del globulo rosso, AORN A. Cardarelli , Naples , Italy.
    Objective: To date in Italy, there is paucity on data about the prevalence, clinical and haematological features of patients carrying the haemoglobin (Hb) Lepore variant in homozygous or in association with other haemoglobinopathies.

    Methods: Here we report the results of a retrospective analysis on 33 patients from Campania, a region of Southern Italy, historically followed at 'UOSD Malattie Rare del Globulo Rosso' of Cardarelli hospital, Naples, Italy.

    Results: We described 33 patients carrying the Hb Lepore variant: 21 compound heterozygotes with a common thalassaemia allele, six patients with homozygous state for Hb Lepore, five patients with Hb Lepore/Hb S and one patient with Hb Lepore/Hb Neapolis were identified. Read More

    Phenotypic and genetic analysis of dysprothrombinemia due to a novel homozygous mutation.
    Hematology 2017 Jul 14;22(6):380-385. Epub 2017 Feb 14.
    a Department of Clinical Laboratory , The First Affiliated Hospital of Wenzhou Medical University , China.
    Objective: We study the phenotype and genotype of a novel gene mutation of factor II (FII) that leads to dysprothrombinemia, and do the meta-analysis to illuminate its molecular pathogenesis. It will further contribute to our comprehension of the pathogenesis of this type of disease.

    Methods: The prothrombin time (PT), activated partial thromboplastin time (APTT) and the activities of other factors were determined by the one-stage clotting method. Read More

    Possible association of 3' UTR +357 A>G, IVS11-nt 93 T>C, c.1311 C>T polymorphism with G6PD deficiency.
    Hematology 2017 Jul 6;22(6):370-374. Epub 2017 Jan 6.
    b Division of Hematology, Department of Internal Medicine , School of Medicine, University of Utah , Salt Lake City , UT , USA.
    Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common X-linked inherited enzymopathic disorder affecting more than 500 million people worldwide. It has so far been linked to 217 distinct genetic variants in the exons and exon-intron boundaries of the G6PD gene, giving rise to a wide range of biochemical heterogeneity and clinical manifestations.

    Objectives: Reports from different settings suggested the association of intronic and other mutations outside the reading frame of the G6PD gene with reduced enzyme activity and presenting clinical symptoms. Read More

    Hb A2 Episkopi - a novel δ-globin chain variant [HBD:c.428C>T] in a family of mixed Cypriot-Lebanese descent.
    Hematology 2017 Jun 22;22(5):304-309. Epub 2016 Dec 22.
    a Department of Molecular Genetics Thalassaemia , The Cyprus Institute of Neurology and Genetics, Cyprus & Cyprus School of Molecular Medicine , Nicosia , Cyprus.
    Objectives: Thalassaemia is a potentially lethal inherited anaemia, caused by reduced or absent synthesis of globin chains. Measurement of the minor adult haemoglobin Hb A2, combining α- with δ-globin, is critical for the routine diagnosis of carrier status for α- or β-thalassaemia. Here, we aim to characterize a novel δ-globin variant, Hb A2 Episkopi, in a single family of mixed Lebanese and Cypriot ancestry with mild hypochromic anaemia and otherwise normal globin genotype, which also presents with a coincidental 0. Read More

    Genetic analysis of a hereditary factor XII deficiency pedigree of a consanguineous marriage due to a homozygous F12 gene mutation: Gly341Arg.
    Hematology 2017 Jun 22;22(5):310-315. Epub 2016 Dec 22.
    a Department of Clinical Laboratory , The First Affiliated Hospital of Wenzhou Medical University , Wenzhou , China.
    Objective And Importance: To study the gene mutations of factor XII (FXII) in a Chinese family of consanguineous marriage with FXII deficiency and illuminate the possible molecular pathogenic mechanism. It will contribute to our comprehension of the pathogenesis of the disease.

    Clinical Presentation: The proband was a 26-year-old Chinese pregnant woman who was discovered, in a pregnancy test, with a prolonged activated partial thromboplastin time (APTT) at 61. Read More

    Investigation of T-cell immunoglobulin- and mucin-domain-containing molecule-3 (TIM-3) polymorphisms in essential thrombocythaemia (ET).
    Hematology 2017 Jul 17;22(6):361-367. Epub 2016 Dec 17.
    a Department of Clinical Laboratory , Shandong Provincial Hospital Affiliated to Shandong University , Jinan , Shandong , China.
    Objectives: T-cell immunoglobulin- and mucin-domain-containing molecule-3 (TIM-3) is preferentially expressed on terminally differentiated Th1 cells and inhibits their IFN-γ production. It has been reported that chronic inflammation may be an important driving force for myeloproliferative neoplasms (MPNs). Therefore, we hypothesized that as an important inflammation regulator, TIM-3 may be involved in essential thrombocythaemia (ET). Read More

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