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    8940 results match your criteria American journal of hematology[Journal]

    1 OF 179

    Mutations and prognosis in myelodysplastic syndromes: Karyotype-adjusted analysis of targeted sequencing in 300 consecutive cases and development of a genetic risk model.
    Am J Hematol 2018 Feb 8. Epub 2018 Feb 8.
    Divisions of Hematology, Mayo Clinic, Rochester, MN, USA.
    In order to develop a genetic risk model for primary myelodysplastic syndromes (MDS), we queried the prognostic significance of next-generation sequencing (NGS)-derived mutations, in the context of the Mayo cytogenetic risk stratification, which includes high-risk (monosomal karyotype; MK), intermediate-risk (non-MK, classified as intermediate/poor/very poor, per the revised international prognostic scoring system; IPSS-R), and low-risk (classified as good/very good, per IPSS-R). Univariate analysis in 300 consecutive patients with primary MDS identified TP53, RUNX1, U2AF1, ASXL1, EZH2 and SRSF2 mutations as "unfavorable" and SF3B1 as "favorable" risk factors for survival; for the purposes of the current study, the absence of SF3B1 mutation was accordingly dubbed as an "adverse" mutation. Analysis adjusted for age and MK, based on our previous observation of significant clustering between MK and TP53 mutations, confirmed independent prognostic contribution from RUNX1, ASXL1 and SF3B1 mutations. Read More

    Comparative safety of intravenous ferumoxytol vs ferric carboxymaltose in iron deficiency anemia: A randomized trial.
    Am J Hematol 2018 Feb 8. Epub 2018 Feb 8.
    AMAG Pharmaceuticals, Inc., Waltham, Massachusetts, USA.
    Few trials have examined rates of hypersensitivity reactions (HSRs) with intravenous iron formulations used to treat iron deficiency anemia. This randomized, multicenter, double-blind clinical trial compared the safety and efficacy of ferumoxytol versus ferric carboxymaltose (FCM), focusing on rates of HSRs and hypotension as the primary end point. Patients with iron deficiency anemia of any etiology in whom oral iron was unsatisfactory or intolerable received ferumoxytol (n = 997) or FCM (n = 1000) intravenously over ≥15 minutes on days 1 and 8 or 9 for total respective doses of 1. Read More

    Chronic myeloid leukemia: 2018 update on diagnosis, therapy and monitoring.
    Am J Hematol 2018 Mar;93(3):442-459
    Department of Leukemia, The University of Texas M. D. Anderson Cancer Center, Houston, Texas.
    Disease Overview: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm with an incidence of 1-2 cases per 100 000 adults. It accounts for approximately 15% of newly diagnosed cases of leukemia in adults.

    Diagnosis: CML is characterized by a balanced genetic translocation, t(9;22)(q34;q11. Read More

    Effectiveness and safety of anticoagulants for the treatment of venous thromboembolism in patients with cancer.
    Am J Hematol 2018 Feb 3. Epub 2018 Feb 3.
    Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio.
    Anticoagulation is used to treat venous thromboembolism (VTE) in cancer patients, but may be associated with an increased risk of bleeding. VTE recurrence and major bleeding were assessed in cancer patients treated for VTE with the most currently prescribed anticoagulants in clinical practice. Newly diagnosed cancer patients (first VTE 1/1/2013-05/31/2015) who initiated rivaroxaban, low-molecular-weight heparin (LMWH), or warfarin were identified from Humana claims data and observed until end of eligibility or end of data availability. Read More

    N-acetyl-L-cysteine Improves Bone Marrow Endothelial Progenitor Cells in Prolonged Isolated Thrombocytopenia Patients post Allogeneic Hematopoietic Stem Cell Transplantation.
    Am J Hematol 2018 Feb 2. Epub 2018 Feb 2.
    Peking University People's Hospital, Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Collaborative Innovation Center of Hematology, Peking University, Beijing, China.
    Prolonged isolated thrombocytopenia (PT) is a serious complication following allogeneic hematopoietic stem cell transplantation (allo-HSCT). According to murine studies, endothelial progenitor cells (EPCs) play a crucial role in the regulation of hematopoiesis and thrombopoiesis in the bone marrow (BM) microenvironment. We previously showed that the reduced frequency of BM EPCs was an independent risk factor for the occurrence of PT following allo-HSCT. Read More

    Flai (fludarabine, cytarabine, idarubicin) plus low-dose gemtuzumab ozogamicin as induction therapy in cd33-positive aml: Final results and long term outcome of a phase ii multicenter clinical trial.
    Am J Hematol 2018 Feb 2. Epub 2018 Feb 2.
    Division of Hematology and SCT, University of Udine, Italy.
    The aim of this prospective clinical trial was to evaluate the efficacy and safety of a combination of Gemtuzumab-Ozogamicin (GO) and FLAI scheme (fludarabine, cytarabine, idarubicin) as a first-line therapy in CD33 positive acute myeloid leukemia (AML). We treated 130 consecutive patients, aged <65, with a median age of 52 years (range, 18-65). FLAI-GO induction regimen included fludarabine (30 mg/sqm) and cytarabine (2 g/sqm) on days 1-5; idarubicin (10 mg/sqm) on days 1, 3, and 5; and GO (3 mg/sqm) on day 6. Read More

    Phenotypes and survival in Erdheim-Chester disease: Results from a 165-patient cohort.
    Am J Hematol 2018 Feb 2. Epub 2018 Feb 2.
    Internal Medicine Department 2, Pitié-Salpêtrière Hospital, French National Centre for Rare Systemic Diseases, AP-HP, Paris, 75013, France.

    Multi-gene panel testing improves diagnosis and management of patients with hereditary anemias.
    Am J Hematol 2018 Feb 3. Epub 2018 Feb 3.
    Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università degli Studi di Napoli Federico II, Napoli, Italy.
    Mutations in more than 70 genes cause hereditary anemias (HA), a highly heterogeneous group of rare/low frequency disorders in which we included: hyporegenerative anemias, as congenital dyserythropoietic anemia (CDA) and Diamond-Blackfan anemia; hemolytic anemias due to erythrocyte membrane defects, as hereditary spherocytosis and stomatocytosis; hemolytic anemias due to enzymatic defects. The study describes the diagnostic workflow for HA, based on the development of two consecutive versions of a targeted-NGS panel, including 34 and 71 genes, respectively. Seventy-four probands from 62 unrelated families were investigated. Read More

    Allogeneic hematopoietic stem cell transplant overcomes the adverse survival effect of very high risk and unfavorable karyotype in myelofibrosis.
    Am J Hematol 2018 Feb 1. Epub 2018 Feb 1.
    Divisions of Hematology, Mayo Clinic, Rochester, MN.
    The prognostic importance of genetic information in primary myelofibrosis (PMF) was recently highlighted in a study of over 1,000 cytogenetically-annotated patients; 5-year survival rates were 8% for very high risk (VHR), 27% "unfavorable" and 45% "favorable" karyotype. The current study addresses the practice-relevant question of whether or not allogeneic hematopoietic stem cell transplant (HCT) can overcome the detrimental survival effect of VHR or unfavorable karyotype. The study included 67 patients with PMF or secondary MF who received HCT at the Mayo Clinic and in whom pre-transplant cytogenetic information was available. Read More

    Glucose-6-phosphate-dehydrogenase deficient red blood cell units are associated with decreased posttransfusion red blood cell survival in children with sickle cell disease.
    Am J Hematol 2018 Jan 27. Epub 2018 Jan 27.
    Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Department of Pediatrics and Hematology/Oncology, Emory University School of Medicine, Atlanta, Georgia.
    Chronic transfusion therapy (CTT) for sickle cell disease (SCD) reduces disease morbidity by suppressing the amount of circulating hemoglobin S (HbS)-containing red blood cells (RBC). The effectiveness of CTT depends on the rate of RBC clearance. Glucose-6-phosphate dehydrogenase (G6PD) deficient donor RBC may exhibit increased hemolysis, but it is unknown if transfusion of these units results in less effective transfusion outcomes in SCD. Read More

    Nationwide survey on the use of horse antithymocyte globulins (ATGAM) in patients with acquired aplastic anemia: A report on behalf of the French Reference Center for Aplastic Anemia.
    Am J Hematol 2018 Jan 27. Epub 2018 Jan 27.
    Service Hématologie Greffe, Centre de Référence Aplasies Médullaires Acquises et Constitutionnelles, Université Paris Diderot, Sorbonne Paris Cité, Inserm UMR 1160, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France.
    Antithymocyte globulins (ATG) plus cyclosporine (CSA) is the gold standard immunosuppressive treatment (IST) for patients with aplastic anemia. A prospective randomized trial showed in 2011 that hATG was superior to rabbit ATG for first-line treatment of severe AA. The French Health Agency (ANSM) permitted a patient-named authorization for temporary use (ATU) program of hATG (ATGAM, Pfizer) in patients with AA in 2011 since commercial access to hATG is not approved. Read More

    Lymphoproliferative disorders in patients with chronic myeloproliferative neoplasms: A systematic review.
    Am J Hematol 2018 Jan 27. Epub 2018 Jan 27.
    Research Foundation FROM, Papa Giovanni XXIII Hospital, Piazza OMS 1, 24127 Bergamo, Italy.
    Patients with a Ph-negative myeloproliferative neoplasm (MPN) may harbor or develop lymphoproliferative disorders (LPD), however, the clinical and molecular determinants of MPN and LPD co-occurrence are still uncertain. To systematically pool the available knowledge, we conducted a scoping review of literature published since January 2005 and analyzed single-patient clinical data from 50 papers reporting 214 individuals harboring both MPN and LPD. Patients had been diagnosed essential thrombocythemia (44%), polycythemia vera (29%), or myelofibrosis (23%) at a median age of 67 years (26-94): half of them incurred a LPD after a median of 72 months from MPN diagnosis, while in 20% the LPD diagnosis was antecedent or synchronous. Read More

    A prospective analysis for prevalence of complications in Thai nontransfusion-dependent Hb E/β-thalassemia and α-thalassemia (Hb H disease).
    Am J Hematol 2018 Jan 23. Epub 2018 Jan 23.
    Division of Hematology/Oncology, Department of Pediatrics, , Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
    Recently, complications in patients with nontransfusion-dependent thalassemia (NTDT), in particular those with β-thalassemia intermedia (β-TI), were found to be significantly different from those in patients with transfusion dependent thalassemia (TDT), mainly β-thalassemia major (β-TM). However, this information is rather limited in other forms of NTDT. In this prospective study, adult Thai NTDT patients were interviewed and clinically evaluated for thalassemia related complications. Read More

    The spleen of patients with myelofibrosis harbors defective mesenchymal stromal cells.
    Am J Hematol 2018 Jan 23. Epub 2018 Jan 23.
    Center for the Study of Myelofibrosis, Laboratory of Biochemistry, Biotechnology and Advanced Diagnosis, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.
    Splenic hematopoiesis is a major feature in the course of myelofibrosis (MF). In fact, the spleen of patients with MF contains malignant hematopoietic stem cells retaining a complete differentiation program, suggesting both a pivotal role of the spleen in maintaining the disease and a tight regulation of hematopoiesis by the splenic microenvironment, in particular by mesenchymal stromal cells (MSCs). Little is known about splenic MSCs (Sp-MSCs), both in normal and in pathological context. Read More

    Band 3 phosphorylation induces irreversible alterations of stored red blood cells.
    Am J Hematol 2018 Jan 20. Epub 2018 Jan 20.
    Université Sorbonne Paris Cité, Université Paris Diderot, Inserm, INTS, Unité Biologie Intégrée du Globule Rouge, Laboratoire d'Excellence GR-Ex, Paris, France.

    Long-term effects of crizotinib in ALK-positive tumors (excluding NSCLC): A phase 1b open-label study.
    Am J Hematol 2018 Jan 20. Epub 2018 Jan 20.
    Seoul National University Hospital, Seoul, South Korea.
    Crizotinib, an inhibitor of anaplastic lymphoma kinase (ALK), MET, and ROS1, is approved for treatment of patients with ALK-positive or ROS1-positive advanced non-small-cell lung cancer (NSCLC). However, ALK rearrangements are also implicated in other malignancies, including anaplastic large-cell lymphoma and inflammatory myofibroblastic tumors (IMTs). In this ongoing, multicenter, single-arm, open-label phase 1b study (PROFILE 1013; NCT01121588), patients with ALK-positive advanced malignancies other than NSCLC were to receive a starting dose of crizotinib 250 mg twice daily. Read More

    A phase II study of tacrolimus and thymoglobulin as graft-versus-host-disease prophylaxis in related donor allogeneic hematopoietic cell transplantation.
    Am J Hematol 2018 Jan 10. Epub 2018 Jan 10.
    Department of Oncology, Blood and Marrow Stem Cell Transplant Program, Karmanos Cancer Institute/Wayne State University, Detroit, Michigan.

    Realizing effectiveness across continents with hydroxyurea: Enrollment and baseline characteristics of the multicenter REACH study in Sub-Saharan Africa.
    Am J Hematol 2018 Jan 10. Epub 2018 Jan 10.
    Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
    Despite its well-described safety and efficacy in the treatment of sickle cell anemia (SCA) in high-income settings, hydroxyurea remains largely unavailable in sub-Saharan Africa, where more than 75% of annual SCA births occur and many comorbidities exist. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, NCT01966731) is a prospective, Phase I/II open-label trial of hydroxyurea designed to evaluate the feasibility, safety, and benefits of hydroxyurea treatment for children with SCA in four sub-Saharan African countries. Read More

    Description and prognostic significance of the kinetics of minimal residual disease status in adults with acute lymphoblastic leukemia treated with HyperCVAD.
    Am J Hematol 2018 Jan 10. Epub 2018 Jan 10.
    Department of Medicine, University of Washington School of Medicine, Seattle, Washington.
    HyperCVAD is a commonly-used regimen for adults with newly-diagnosed acute lymphoblastic leukemia (ALL). However, relatively little is known about the application of minimal residual disease (MRD) detection with this treatment. To address this, we studied 142 adults with ALL treated with hyperCVAD over a 10-year period who had MRD assessed by either multi-parameter flow cytometry or (for patients with Philadelphia chromosome positive ALL) reverse transcriptase polymerase chain reaction for the BCR-ABL1 translocation. Read More

    Follicular lymphoma: 2018 update on diagnosis and management.
    Am J Hematol 2018 Feb;93(2):296-305
    Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts.
    Disease Overview: Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma (FL) is characterized by diffuse lymphadenopathy, bone marrow involvement, splenomegaly and less commonly other extranodal sites of involvement. In general, cytopenias can occur but constitutional symptoms of fever, nightsweats, and weight loss are uncommon. Read More

    Toward complement inhibition 2.0: Next generation anticomplement agents for paroxysmal nocturnal hemoglobinuria.
    Am J Hematol 2018 Jan 4. Epub 2018 Jan 4.
    Hematology, Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy.
    Therapeutic complement inhibition by eculizumab has revolutionized the treatment of paroxysmal nocturnal hemoglobinuria (PNH) with a major impact on its natural history. Nevertheless, emerging unmet clinical needs may benefit from the development of novel complement inhibitors. Novel strategies of complement inhibition exploit different agents targeting C5, as well as compound intercepting the complement cascade at the level of its key component C3, or even upstream at the level of components involved in complement alternative pathway initiation. Read More

    Centrifugation-free washing: A novel approach for removing immunoglobulin A from stored red blood cells.
    Am J Hematol 2017 Dec 29. Epub 2017 Dec 29.
    Department of Biomedical Engineering, University of Houston, Houston, Texas 77204.
    Washed red blood cells (RBCs) are indicated for immunoglobulin A (IgA) deficient recipients. Centrifugation-based cell processors commonly used by hospital blood banks cannot consistently reduce IgA below the recommended levels, hence double washing is frequently required. Here, we describe a prototype of a simple, portable, disposable system capable of washing stored RBCs without centrifugation, while reducing IgA below 0. Read More

    Ataluren-driven restoration of Shwachman-Bodian-Diamond syndrome protein function in Shwachman-Diamond syndrome bone marrow cells.
    Am J Hematol 2017 Dec 29. Epub 2017 Dec 29.
    Cystic Fibrosis Center, Azienda Ospedaliero Universitaria Ospedali Riuniti, Ancona, Italy.
    Shwachman-Diamond syndrome (SDS) is a rare inherited recessive disease mainly caused by mutations in the Shwachman-Bodian-Diamond syndrome (SBDS) gene, which encodes for the homonymous protein SBDS, whose function still remains to be fully established. SDS affects several organs causing bone marrow failure, exocrine pancreatic insufficiency, skeletal malformations, and cognitive disorders. About 15% of SDS patients develop myelodysplastic syndrome (MDS) and are at higher risk of developing acute myeloid leukemia (AML). Read More

    FLT3 inhibitors in acute myeloid leukemia: Choosing the best when the optimal does not exist.
    Am J Hematol 2017 Dec 29. Epub 2017 Dec 29.
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Despite significant advances in deciphering the molecular and cytogenetic pathways governing acute myeloid leukemia, improvements in treatment strategies and clinical outcomes have been limited. The discovery of FLT3 pathway and its potential role in leukemogenesis has generated excitement in the field and has provided a potential target for drug development. Despite setbacks encountered with first-generation inhibitors, we are witnessing an outbreak of novel agents with potent activity and improved pharmacodynamics which continue to generate promising results. Read More

    Ibrutinib discontinuation in Waldenström macroglobulinemia: Etiologies, outcomes, and IgM rebound.
    Am J Hematol 2017 Dec 27. Epub 2017 Dec 27.
    Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Boston, Massachusetts.
    Ibrutinib is the first approved therapy for symptomatic patients with Waldenström macroglobulinemia (WM). The reasons for discontinuing ibrutinib and subsequent outcomes have not been previously evaluated in WM patients. We therefore conducted a retrospective review of 189 WM patients seen at our institution who received treatment with ibrutinib, of whom 51 (27%) have discontinued therapy. Read More

    JAK2 exon 12 mutated polycythemia vera: Mayo-Careggi MPN Alliance study of 33 consecutive cases and comparison with JAK2V617F mutated disease.
    Am J Hematol 2017 Dec 23. Epub 2017 Dec 23.
    Department of Experimental and Clinical Medicine, CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliera Universitaria Careggi, University of Florence, Florence, Italy.

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