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    8787 results match your criteria American journal of hematology[Journal]

    1 OF 176

    Treatment of hepatitis C virus infection with direct-acting antiviral drugs is safe and effective in patients with hemoglobinopathies.
    Am J Hematol 2017 Sep 20. Epub 2017 Sep 20.
    Centro della Microcitemia e delle Anemie Congenite, Galliera Hospital, Genoa, Italy.
    Progression of liver fibrosis in patients with hemoglobinopathies is strongly related to the severity of iron overload and the presence of chronic hepatitis C virus (HCV) infection. Effective iron chelation therapy and HCV infection eradication may prevent liver complications. The European Association for the Study of the Liver guidelines recommend interferon-free regimens for the treatment of HCV infection in patients with hemoglobinopathies. Read More

    Effects of Pre- and Post-transplantation Minimal Residual Disease on Outcomes in Pediatric Patients with Acute Myeloid Leukemia Receiving Human Leukocyte Antigen-matched or Mismatched Related Donor Allografts.
    Am J Hematol 2017 Sep 20. Epub 2017 Sep 20.
    Peking University People's Hospital & Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, No. 11 South Street of Xizhimen, Xicheng District, Beijing 100044, P.R.C.

    Incidence and predictive score for delayed hemolytic transfusion reaction in adult patients with sickle cell disease.
    Am J Hematol 2017 Sep 19. Epub 2017 Sep 19.
    Etablissement Français du Sang, Créteil, 94000, France.
    Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication of transfusion in sickle cell disease (SCD). The frequency of DHTR is underestimated because its symptoms mimic those of vaso-occlusive crisis and antibodies (Abs) are often not detectable. No predictive factors for identifying patients likely to develop DHTR have yet been defined. Read More

    A Clinically Meaningful Fetal Hemoglobin Threshold for Children with Sickle Cell Anemia During Hydroxyurea Therapy.
    Am J Hematol 2017 Sep 14. Epub 2017 Sep 14.
    Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, USA.
    Hydroxyurea has proven clinical benefits and is recommended to be offered to all children with sickle cell anemia (SCA), but the optimal dosing regimen remains controversial. Induction of red blood cell fetal hemoglobin (HbF) by hydroxyurea appears to be dose-dependent. However, it is unknown whether maximizing HbF% improves clinical outcomes. Read More

    A nationwide survey of hypoplastic myelodysplastic syndrome (a multicenter retrospective study).
    Am J Hematol 2017 Sep 11. Epub 2017 Sep 11.
    Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
    Hypoplastic myelodysplastic syndrome (hMDS) is a distinct entity with bone marrow (BM) hypocellularity and the risk of death from BM failure (BMF). To elucidate the characteristics of hMDS, the data of 129 patients diagnosed between April 2003 and March 2012 were collected from 20 institutions and the central review team of the National Research Group on Idiopathic Bone Marrow Failure Syndromes, and compared with 115 non-hMDS patients. More RA and fewer CMMoL and RAEB-t in French-American-British (FAB) and more RCUD and MDS-U and fewer RCMD in World Health Organization (WHO) classifications were found in hMDS than non-hMDS with significant differences. Read More

    Targeted next-generation sequencing in myelodysplastic syndromes and prognostic interaction between mutations and IPSS-R.
    Am J Hematol 2017 Sep 5. Epub 2017 Sep 5.
    Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
    A 27-gene panel was used for next-generation sequencing (NGS) in 179 patients (median age 73 years) with primary myelodysplastic syndromes (MDS); risk distribution according to the revised International Prognostic Scoring System (IPSS-R) was 11% very high, 18% high, 17% intermediate, 38% low and 16% very low. At least one mutation/variant was detected in 147 (82%) patients; 23% harbored three or more mutations/variants. The most frequent mutations/variants included ASXL1 (30%), TET2 (25%), SF3B1 (20%), U2AF1 (16%), SRSF2 (16%), TP53 (13%), RUNX1 (11%) and DNMT3A (10%). Read More

    Cutaneous T-cell lymphoma: 2017 update on diagnosis, risk-stratification, and management.
    Am J Hematol 2017 Oct;92(10):1085-1102
    Division of Hematology/Oncology, University of Michigan Comprehensive Cancer Center, Ann Arbor, Michigan, 48109-5948.
    Disease Overview: Cutaneous T-cell lymphomas are a heterogenous group of T-cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS).

    Diagnosis: The diagnosis of MF or SS requires the integration of clinical and histopathologic data.

    Risk-adapted Therapy: TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a "risk-adapted," multi-disciplinary approach to treatment. Read More

    Long-term survival of beta thalassemia major patients treated with hematopoietic stem cell transplantation compared with survival with conventional treatment.
    Am J Hematol 2017 Aug 29. Epub 2017 Aug 29.
    Bone Marrow Transplant Center, Hospital "Binaghi & Microcitemico", Cagliari, Italy.
    Allogeneic hematopoietic stem cell transplantation (HSCT) in thalassemia remains a challenge. We reported a single-centre case-control study of a large cohort of 516 children and adult patients treated with HSCT or blood transfusion support and iron chelation therapy; 258 patients (median age 12, range 1-45) underwent sibling (67%) or unrelated (33%) HSCT; 97 patients were adults (age ≥ 16 years). The median follow-up after HSCT was 11 years (range 1-30). Read More

    Clinical Outcomes in Adult Patients with Aplastic Anemia- a single institution experience.
    Am J Hematol 2017 Aug 29. Epub 2017 Aug 29.
    Departments of Leukemia, The University of Texas, M. D. Anderson Cancer Center, Houston, Texas.
    Newer treatment modalities are being investigated to improve upon historical outcomes with standard immunosuppressive therapy (IST) in aplastic anemia (AA). We analyzed outcomes of adult patients with AA sequentially treated with various combinatorial anti-thymoglobulin-based IST regimens in frontline and relapsed/refractory (R/R) settings. Pretreatment and on-treatment clinical characteristics were analyzed for relationships to response and outcome. Read More

    A Single Center Phase II Study of Ixazomib in Patients with Relapsed or Refractory Cutaneous or Peripheral T-cell Lymphomas.
    Am J Hematol 2017 Aug 26. Epub 2017 Aug 26.
    Department of Internal Medicine, Division of Hematology and Oncology, University of Michigan, Ann Arbor, MI.
    The transcription factor GATA-3, highly expressed in many cutaneous and peripheral T-cell lymphomas, confers resistance to chemotherapy in a cell-autonomous manner. As GATA-3 is transcriptionally regulated by NF-κB, we sought to determine the extent to which proteasomal inhibition impairs NF-κB activation and GATA-3 expression and cell viability in malignant T cells. Proteasome inhibition, NF-κB activity, GATA-3 expression, and cell viability were examined in patient-derived cell lines and primary T-cell lymphoma specimens ex vivo treated with the oral proteasome inhibitor ixazomib. Read More

    Changes in intestinal microbiota and their effects on allogeneic stem cell transplantation.
    Am J Hematol 2017 Aug 26. Epub 2017 Aug 26.
    Division of Hematology and Oncology, Rhode Island Hospital/Brown University Warren Alpert School of Medicine, Providence, Rhode Island.
    The human intestinal microbiota is essential for microbial homeostasis, regulation of metabolism, and intestinal immune tolerance. Rapidly evolving understanding of the importance of the microbiota implicates changes in the composition and function of intestinal microbial communities in an assortment of systemic conditions. Complications following allogeneic stem cell transplant now join the ever-expanding list of pathologic states regulated by intestinal microbiota. Read More

    Comprehensive evaluation of the revised international staging system in multiple myeloma patients treated with novel agents as a primary therapy.
    Am J Hematol 2017 Aug 22. Epub 2017 Aug 22.
    Division of Hematology/Oncology, Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul, South Korea.
    The revised International Staging System (R-ISS) has recently been developed to improve the risk stratification of multiple myeloma (MM) patients over the ISS. We assessed the R-ISS in MM patients who were treated with novel agents as a primary therapy and evaluated its discriminative power and ability to reclassify patients from the ISS. A total of 514 newly diagnosed MM patients treated with novel agents including thalidomide, bortezomib, and lenalidomide as a primary therapy were included in this retrospective analysis. Read More

    First-line treatment selection and early monitoring patterns in chronic phase-chronic myeloid leukemia in routine clinical practice: SIMPLICITY.
    Am J Hematol 2017 Aug 17. Epub 2017 Aug 17.
    Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, Box 489, New York, NY 10065, USA.
    Achieving successful outcomes in chronic phase-chronic myeloid leukemia (CP-CML) requires careful monitoring of cytogenetic/molecular responses (CyR/MR). SIMPLICITY (NCT01244750) is an observational study exploring tyrosine kinase inhibitor use and management patterns in patients with CP-CML receiving first-line imatinib (n=416), dasatinib (n=418) or nilotinib (n=408) in the US and 6 European countries in routine clinical practice. Twelve-month follow-up data of 1,242 prospective patients (enrolled October 01 2010-September 02 2015) are reported. Read More

    A distinctive histidine residue is essential for in vivo glycation-inactivation of human CD59 transgenically expressed in mice erythrocytes: Implications for human diabetes complications.
    Am J Hematol 2017 Aug 17. Epub 2017 Aug 17.
    Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
    Clinical and experimental evidences support a link between the complement system and the pathogenesis of diabetes complications. CD59, an extracellular cell membrane-anchored protein, inhibits formation of the membrane attack complex (MAC), the main effector of complement-mediated tissue damage. This complement regulatory activity of human CD59 (hCD59) is inhibited by hyperglycemia-induced ɛ-amino glycation of Lys(41) . Read More

    Treatment of Individuals Who Cannot Receive Blood Products for Religious or Other Reasons.
    Am J Hematol 2017 Aug 17. Epub 2017 Aug 17.
    Division of Hematology and Medical Oncology, Oregon Health and Science University, Knight Cancer Institute, Portland, Oregon.
    By virtue of their religious principles, Jehovah's Witnesses (JWs) generally object to receiving blood products, raising numerous ethical, legal, and medical challenges for providers who care for these patients, especially in the emergent setting. In this review, we discuss several areas relevant to the care of JWs, including the current literature on "bloodless" medical care in the setting of peri- and intra-operative management, acute blood loss, trauma, pregnancy, and malignancy. We have found that medical and administrative efforts in the form of bloodless medicine and surgery programs can be instrumental in helping to reduce risks of morbidity and mortality in these patients. Read More

    Bmp2 controls iron homeostasis in mice independent of Bmp6.
    Am J Hematol 2017 Aug 17. Epub 2017 Aug 17.
    Program in Anemia Signaling Research, Division of Nephrology, Program in Membrane Biology, Center for Systems Biology, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
    Hepcidin is a key iron regulatory hormone that controls expression of the iron exporter ferroportin to increase the iron supply when needed to support erythropoiesis and other essential functions, but to prevent the toxicity of iron excess. The bone morphogenetic protein (BMP)-SMAD signaling pathway, through the ligand BMP6 and the co-receptor hemojuvelin, is a central regulator of hepcidin transcription in the liver in response to iron. Here, we show that dietary iron loading has a residual ability to induce Smad signaling and hepcidin expression in Bmp6-/- mice, effects that are blocked by a neutralizing BMP2/4 antibody. Read More

    Efficacy of daratumumab-based therapies in patients with relapsed, refractory multiple myeloma treated outside of clinical trials.
    Am J Hematol 2017 Aug 11. Epub 2017 Aug 11.
    Division of Hematology, Mayo Clinic, Rochester, Minnesota.
    Outside of clinical trials, experience with daratumumab-based combination therapies (DCTs) using bortezomib (V)/lenalidomide (R)/pomalidomide (P), and dexamethasone (d) in relapsed/refractory multiple myeloma (RRMM) is limited. We reviewed the outcomes of 126 patients who received ≥ 1 cycle of any DCT. Median age at DCT initiation was 67 (range, 43-93) years. Read More

    Gender and survival in essential thrombocythemia: A two-center study of 1,494 patients.
    Am J Hematol 2017 Aug 10. Epub 2017 Aug 10.
    Department of Experimental and Clinical Medicine, CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliera Universitaria Careggi, University of Florence, Florence, Italy.
    Based on suggestive information from recent epidemiologic data and earlier retrospective studies, we revisited the effect of gender on survival in 1,494 patients with essential thrombocythemia (ET). The primary study population included 904 patients from the Mayo Clinic (median age 58 years; 65% females); risk distribution, according to the international prognostic score for ET (IPSET), was 23% high, 42% intermediate and 35% low. Multivariable analysis that included IPSET-relevant risk factors identified male sex (HR 1. Read More

    Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment.
    Am J Hematol 2017 Sep;92(9):946-965
    Department I of Internal Medicine, Center for Integrated Oncology Köln Bonn, Center of Excellence on "Cellular Stress Responses in Aging-Associated Diseases," University of Cologne, Kerpener Strasse 62, Köln, 50937, Germany.
    Disease Overview: Chronic lymphocytic leukemia (CLL) is the commonest leukemia in western countries. The disease typically occurs in elderly patients and has a highly variable clinical course. Leukemic transformation is initiated by specific genomic alterations that impair apoptosis of clonal B cells. Read More

    Risk factors and a prognostic model for postsplenectomy survival in myelofibrosis.
    Am J Hematol 2017 Aug 7. Epub 2017 Aug 7.
    Department of, Surgery, Mayo Clinic, Rochester, Minnesota.
    Palliative treatment in myelofibrosis (MF) includes transfusion support, JAK2 inhibitors, involved field radiotherapy and splenectomy. To assist in selecting patients who are likely to benefit from splenectomy, we looked into risk factors for postsplenectomy survival, in 120 consecutive cases (median age 66 years); at the time of splenectomy, 61% displayed red cell transfusion need, 49% platelet count <100 × 10(9)/L, 25% leukocyte count >25 × 10(9)/L, 60% constitutional symptoms and 13% circulating blasts ≥5%; dynamic international prognostic scoring system risk categories were 21% high, 55% intermediate-2, 21% intermediate-1 and 3% low. Among informative cases, karyotype was abnormal in 60% and driver mutational status was JAK2 75%, CALR 15%, MPL 4% and triple-negative 6%. Read More

    SIRT1 activates the expression of fetal hemoglobin genes.
    Am J Hematol 2017 Aug 4. Epub 2017 Aug 4.
    Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, 02118.
    High fetal hemoglobin (HbF, α2 γ2 ) levels ameliorate the clinical manifestations of sickle cell disease and β thalassemia. The mechanisms that repress HbF expression and silence γ-globin genes in adults are incompletely characterized and only a single HbF inducer, hydroxyurea, is approved for treatment, and only in patients with sickle cell disease. We identified SIRT1, a protein deacetylase, as a new inducer of γ-globin. Read More

    The impact of antibody profile in thrombosis associated with primary antiphospholipid syndrome.
    Am J Hematol 2017 Aug 1. Epub 2017 Aug 1.
    Thrombosis and Haemostasis Unit, Hematology and Hemotherapy Center, University of Campinas, Rua Carlos Chagas, 480. Cidade Universitária "ZeferinoVaz" CEP: 13083-970, Campinas, SP, Brazil.
    Triple positivity (TP) for antiphospholipid antibodies(aPL) may identify aPL carriers with poorer prognosis. The clinical impact of TP in primary antiphospholipid syndrome(PAPS) remains unclear and further clinical evidences are needed to validate TP as a marker of severity. The aim of this study was to evaluate the impact of TP on the clinical course of PAPS with thrombosis(t-PAPS). Read More

    Outcomes after 18 Months of Eliglustat Therapy in Treatment-Naïve Adults with Gaucher Disease Type 1: The Phase 3 ENGAGE Trial.
    Am J Hematol 2017 Aug 1. Epub 2017 Aug 1.
    Sanofi Genzyme, Cambridge, MA, USA.
    Eliglustat, an oral substrate reduction therapy, is a first-line treatment for adults with Gaucher disease type 1 (GD1) who are poor, intermediate, or extensive CYP2D6 metabolizers (>90% of patients). In the primary analysis of the Phase 3 ENGAGE trial (NCT00891202), eliglustat treatment for 9 months resulted in significant reductions in spleen and liver volumes and increases in hemoglobin concentration and platelet count compared with placebo. We report 18-month outcomes of patients who entered the trial extension period, in which all patients received eliglustat. Read More

    Plasmapheresis may improve clinical condition in sickle cell disease through its effects on red blood cell rheology.
    Am J Hematol 2017 Jul 25. Epub 2017 Jul 25.
    Cardeza Foundation, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania.

    Fetal hemoglobin in sickle cell anemia: The Arab-Indian haplotype and new therapeutic agents.
    Am J Hematol 2017 Jul 24. Epub 2017 Jul 24.
    Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, 02118.
    Fetal hemoglobin (HbF) has well-known tempering effects on the symptoms of sickle cell disease and its levels vary among patients with different haplotypes of the sickle hemoglobin gene. Compared with sickle cell anemia haplotypes found in patients of African descent, HbF levels in Saudi and Indian patients with the Arab-Indian (AI) haplotype exceed that in any other haplotype by nearly twofold. Genetic association studies have identified some loci associated with high HbF in the AI haplotype but these observations require functional confirmation. Read More

    Frequency, risk factors, and outcomes of central nervous system relapse in lymphoma patients treated with dose-adjusted EPOCH plus rituximab.
    Am J Hematol 2017 Jul 18. Epub 2017 Jul 18.
    Division of Hematology, Mayo Clinic, Rochester, Minnesota.
    Background: Central nervous system (CNS) relapse in non-Hodgkin lymphoma (NHL) is a rare but serious complication that carries a poor prognosis. The use of infusional etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (EPOCH-R) for frontline treatment of diffuse large B cell lymphoma (DLBCL) is increasing, though little is known about incidence of and risk factors for CNS relapse with this regimen PATIENTS AND METHODS: We completed a chart review of patients with NHL who received EPOCH-R as front line therapy. Data obtained included baseline and treatment characteristics including if patients received CNS directed therapy. Read More

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