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    8852 results match your criteria American journal of hematology[Journal]

    1 OF 178

    Ruxolitinib-associated infections: a systematic review and meta-analysis.
    Am J Hematol 2017 Nov 18. Epub 2017 Nov 18.
    Research Center on Thromboembolic Disorders and Antithrombotic Therapies, Department of Clinical and Experimental Medicine, University of Insubria, Varese, Italy.
    Ruxolitinib exerts immunosuppressive activity that may increase the risk of infectious complications. We performed a systematic review of the literature with the aim of estimating the risk of infections in patients treated with ruxolitinib. Studies were identified by electronic search of MEDLINE and EMBASE database. Read More

    Impact of gene mutations on treatment response and prognosis of acute myeloid leukemia secondary to myeloproliferative neoplasms.
    Am J Hematol 2017 Nov 17. Epub 2017 Nov 17.
    Institut Paoli-Calmettes (IPC), Département d'Hématologie.
    Acute myeloid leukemias secondary (sAML) to myeloproliferative neoplasms (MPN) have variable clinical courses and outcomes, but remain almost always fatal. Large cohorts of sAML to MPN are difficult to obtain and there is very little scientific literature or prospective trials for determining robust prognostic markers and efficient treatments. We analyzed event-free survival (EFS) and overall survival (OS) of 73 patients with MPN who progressed to sAML, based on their epidemiological characteristics, the preexisting MPN, the different treatments received, the different prognostic groups and the responses achieved according to the ELN, and their mutational status determined by next-generation DNA sequencing (NGS). Read More

    Phase 1 Study of Quizartinib in Combination With Induction and Consolidation Chemotherapy in Patients With Newly Diagnosed Acute Myeloid Leukemia.
    Am J Hematol 2017 Nov 15. Epub 2017 Nov 15.
    Leukemia Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, NY.
    Novel therapies have potential to improve outcomes in patients with acute myeloid leukemia (AML) harboring FLT3-ITD mutations that have high risk of relapse and poor survival following standard of care (SOC) cytarabine/anthracycline-based induction/consolidation chemotherapy. Quizartinib is a selective and highly potent FLT3 inhibitor that has shown strong single-agent activity in relapsed or refractory (R/R) AML. This phase 1, open-label, sequential group dose-escalation trial (NCT 01390337) is the first evaluating safety and tolerability of quizartinib in combination with SOC chemotherapy in newly diagnosed AML (ndAML). Read More

    A Phase II Trial of Ruxolitinib in Combination with Azacytidine in Myelodysplastic Syndrome/Myeloproliferative Neoplasms.
    Am J Hematol 2017 Nov 14. Epub 2017 Nov 14.
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
    Ruxolitinib and azacytidine target distinct disease manifestations of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPNs). Patients with MDS/MPNs initially received ruxolitinib BID (doses based on platelets count), continuously in 28-day cycles for the first 3 cycles. Azacytidine 25 mg/m(2) (Day 1-5) intravenously or subcutaneously was recommended to be added to each cycle starting cycle 4 and could be increased to 75 mg/m(2) (Days 1-5) for disease control. Read More

    A key role for Rac and Pak signaling in Neutrophil Extracellular Traps (NETs) formation defines a new potential therapeutic target.
    Am J Hematol 2017 Nov 10. Epub 2017 Nov 10.
    Division of Hematology/Oncology, Boston Children's Hospital, Boston, MA.
    NET formation in mice (NETosis) is supported by reactive oxygen species (ROS) production by NADPH oxidase and histone hypercitrullination by peptidylarginine deiminase 4 (PAD4). Rac1 and Rac2, expressed in polymorphonuclear neutrophils (PMNs), regulate the cytoskeleton, cell shape, adhesion and migration and are also essential components of the NADPH oxidase complex. We aimed to explore the role of the Rac signaling pathway including the upstream guanosine exchange factor (GEF) activator, Vav, and a downstream effector, the p21-activated kinase, Pak, on NETosis in PMNs using a previously described flow-cytometry-based assay. Read More

    A phase I study of lenalidomide plus chemotherapy with mitoxantrone, etoposide, and cytarabine for the reinduction of patients with acute myeloid leukemia.
    Am J Hematol 2017 Nov 9. Epub 2017 Nov 9.
    University of Virginia, Charlottesville, Virginia.
    Patients with relapsed AML have a poor prognosis and limited responses to standard chemotherapy. Lenalidomide is an immunomodulatory drug that may modulate anti-tumor immunity. We performed a study to evaluate the safety and tolerability of lenalidomide with mitoxantrone, etoposide and cytarabine (MEC) in relapsed/refractory AML. Read More

    Safety and Efficacy of Early Start of Iron Chelation Therapy with Deferiprone in Young Children Newly Diagnosed with Transfusion-Dependent Thalassemia: A Randomized Controlled Trial.
    Am J Hematol 2017 Nov 9. Epub 2017 Nov 9.
    ApoPharma Inc., Toronto, Canada.
    Iron overload is inevitable in patients who are transfusion dependent. In young children with transfusion-dependent thalassemia (TDT), current practice is to delay the start of iron chelation therapy due to concerns over toxicities, which have been observed when deferoxamine was started too early. However, doing so may increase the risk of iron accumulation that will be manifested as toxicities later in life. Read More

    Excess mortality among 10-year survivors of classical Hodgkin lymphoma in adolescents and young adults.
    Am J Hematol 2017 Nov 8. Epub 2017 Nov 8.
    Division of Hematology/Oncology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama.
    Adolescents and young adults (AYA) surviving classical Hodgkin lymphoma (cHL) risk long term fatal treatment-related toxicities. We utilized the Surveillance, Epidemiology and End Results (SEER) program to compare excess mortality rate (EMR-observed minus expected mortality) for 10-year survivors of AYA cHL diagnosed in 1973-1992 and 1993-2003 eras. The 15-year EMR reduced from 4. Read More

    Donor age determines outcome in acute leukemia patients over 40 undergoing haploidentical hematopoietic cell transplantation.
    Am J Hematol 2017 Nov 8. Epub 2017 Nov 8.
    Chaim Sheba Medical Center, Hematology Division, Tel Aviv University, Tel-Hashomer, Israel.
    Haploidentical hematopoietic cell transplantation (haplo-HCT) is being increasingly used in acute leukemia patients as an alternative transplant modality when matched sibling or matched unrelated donors are unavailable. As several potential haploidentical relative donors are typically available for a given patient, optimizing donor selection to improve clinical outcome is crucial. The impact of donor age and kinship on the outcome of acute leukemia patients is not clearly established in this setting. Read More

    Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment.
    Am J Hematol 2017 Dec;92(12):1382-1390
    Laboratoire Hématologie, CHU Caen, 14 033, Caen Cedex, France.
    Disease Overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders, characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment.

    Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation.

    Risk Stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood and the immunoglobulin heavy chain variable region gene mutational status. Read More


    Outgrowing the laboratory diagnosis of type 1 von Willebrand disease: A two decade study.
    Am J Hematol 2017 Nov 3. Epub 2017 Nov 3.
    Department of Medicine, Rochester General Hospital, Rochester, New York.
    Von Willebrand Factor (VWF) levels are known to increase with age in the general population, but that effect is unclear in von Willebrand disease (VWD) patients. Thus, it is important to assess the trends of VWF levels with age, and the extent and rate of their normalization in patients with VWD. In a retrospective cohort study, we reviewed the medical records of 126 patients between 1996 and 2016 who met the NHLBI diagnostic criteria for type 1 VWD or "Low VWF" (LVWF). Read More

    Mutations of the integrin αIIb/β3 intracytoplasmic salt bridge cause macrothrombocytopenia and enlarged platelet α-granules.
    Am J Hematol 2017 Nov 1. Epub 2017 Nov 1.
    Institut Hospitalo-Universitaire de Rythmologie et de Modélisation Cardiaque, Plateforme Technologique d'Innovation Biomédicale, Hôpital Xavier Arnozan, Pessac, France.
    Rare gain-of-function mutations within the ITGA2B or ITGB3 genes have been recognized to cause macrothrombocytopenia (MTP). Here we report three new families with autosomal dominant (AD) MTP, two harboring the same mutation of ITGA2B, αIIbR995W, and a third family with an ITGB3 mutation, β3D723H. In silico analysis shows how the two mutated amino acids directly modify the salt bridge linking the intra-cytoplasmic part of αIIb to β3 of the integrin αIIbβ3. Read More

    Demographics and patient characteristics of 1209 patients with Gaucher Disease: Descriptive analysis from the Gaucher Outcome Survey (GOS).
    Am J Hematol 2017 Nov 1. Epub 2017 Nov 1.
    Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil.
    The Gaucher Outcome Survey (GOS) is an international Gaucher disease (GD) registry established in 2010 for patients with a confirmed GD diagnosis, regardless of GD type or treatment status, designed to evaluate the safety and long-term effectiveness of velaglucerase alfa and other GD-related treatments. As of February 25, 2017, 1209 patients had enrolled, the majority from Israel (44.3%) and the US (31. Read More

    Results of a phase 1 study of quizartinib as maintenance therapy in subjects with acute myeloid leukemia in remission following allogeneic hematopoietic stem cell transplant.
    Am J Hematol 2017 Nov 1. Epub 2017 Nov 1.
    Northwestern University, Chicago, Illinois.
    FLT3-ITD-mutated acute myeloid leukemia (AML) has very high risk of relapse and is associated with poor outcome following allogeneic hematopoietic-cell transplant (allo-HCT). This two-part, phase 1, multicenter, open-label, sequential-group, dose-escalation study aimed to determine dose-limiting toxicities (DLTs), maximum tolerated dose (MTD), and safety/tolerability of quizartinib, a selective and highly potent FLT3 inhibitor, when administered as maintenance therapy after allo-HCT. Thirteen subjects with documented FLT3-ITD-mutated AML in morphological remission following allo-HCT received one of two quizartinib dihydrochloride dose levels (DL): 40 mg/d (DL1; n = 7) and 60 mg/d (DL2; n = 6), administered orally in 28-day cycles for up to 24 cycles. Read More


    MYD88 mutation status does not impact overall survival in Waldenström macroglobulinemia.
    Am J Hematol 2017 Oct 28. Epub 2017 Oct 28.
    Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.
    Waldenström macroglobulinemia (WM) is an immunoglobulin M-associated lymphoma, with majority of cases demonstrating MYD88 locus alteration, most commonly, MYD88(L265P) . Owing to low prevalence of the wild-type (WT) MYD88 genotype in WM, clinically relevant data in this patient population are sparse, with one study showing nearly a 10-fold increased risk of mortality in this subgroup compared to patients with MYD88(L265P) mutation. We studied a large cohort of patients with MYD88(L265P) and MYD88(WT) WM, evaluated at Mayo Clinic, Rochester, between 1995 and 2016, to specifically assess the impact of these genotypes on clinical course. Read More


    Efficacy of VDT PACE-like regimens in treatment of relapsed/refractory multiple myeloma.
    Am J Hematol 2017 Oct 25. Epub 2017 Oct 25.
    Division of Hematology, Mayo Clinic, Rochester, Minnesota.
    Experience with intensive chemotherapy for relapsed/refractory multiple myeloma (RRMM) using VDT PACE regimen and its modifications (VDT PACE-like regimens: VPLRs) outside TOTAL THERAPY trials is limited. We analyzed the outcomes of 141 patients with RRMM who received VPLRs at our center between 2006 and 2017 in an intent-to-treat analysis. Median age was 59. Read More


    Donor lymphocyte infusion and methotrexate for immune recovery after T-cell depleted haploidentical transplantation.
    Am J Hematol 2017 Oct 19. Epub 2017 Oct 19.
    Biostatistics, University of North Carolina - Chapel Hill, North Carolina.
    CD34+ cell selection minimizes graft-versus-host disease (GVHD) after haploidentical donor stem cell transplant but is associated with slow immune recovery and infections. We report a Phase I/II study of prophylactic donor lymphocyte infusion (DLI) followed by methotrexate (MTX) GVHD prophylaxis after CD34-selected haploidentical donor transplant. A prophylactic DLI was given between day +30 and +42. Read More

    Hyper-CVAD plus nelarabine in newly diagnosed adult T-cell acute lymphoblastic leukemia and T-lymphoblastic lymphoma.
    Am J Hematol 2017 Oct 19. Epub 2017 Oct 19.
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Nelarabine, a water soluble prodrug of 9-β-D-arabinofuranosylguanine (ara-G), is a T-cell specific purine nucleoside analogue. Given its activity in relapsed and refractory T acute lymphoblastic leukemia (T-ALL) and T lymphoblastic lymphoma (T-LBL), we sought to define its role in the frontline treatment of adult patients. Therefore, we conducted a single arm phase 2 study to determine the safety and efficacy of nelarabine in combination with hyper-CVAD in newly diagnosed patients. Read More

    A randomized controlled trial comparing two vaso-occlusive episode (VOE) protocols in sickle cell disease (SCD).
    Am J Hematol 2017 Oct 19. Epub 2017 Oct 19.
    Hematology and Medical Oncology Associate Director of The Mount Sinai Comprehensive Sickle Cell Program Icahn School of Medicine at Mount Sinai.
    Limited evidence guides opioid dosing strategies for acute Sickle Cell (SCD) pain. We compared two National Heart, Lung and Blood (NHBLI) recommended opioid dosing strategies (weight-based vs. patient-specific) for ED treatment of acute vaso-occlusive episodes (VOE). Read More

    MECOM, HBS1L-MYB, THRB-RARB, JAK2, and TERT polymorphisms defining the genetic predisposition to myeloproliferative neoplasms: A study on 939 patients.
    Am J Hematol 2017 Oct 19. Epub 2017 Oct 19.
    Department of Medical Genetics, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
    Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) are classical myeloproliferative neoplasms (MPN), characterized by specific somatic mutations in JAK2, CALR or MPL genes. JAK2 46/1 and TERT rs2736100 polymorphisms are known to significantly predispose to MPN. This study aimed to establish the additional contribution of the recently described MECOM rs2201862, HBS1L-MYB rs9376092 and THRB-RARB rs4858647 polymorphisms to the occurrence of MPN. Read More

    World Health Organization-defined eosinophilic disorders: 2017 update on diagnosis, risk stratification, and management.
    Am J Hematol 2017 Nov;92(11):1243-1259
    Stanford Cancer Institute, Stanford, California 94305-5821.
    Disease Overview: The eosinophilias encompass a broad range of nonhematologic (secondary or reactive) and hematologic (primary, clonal) disorders with potential for end-organ damage.

    Diagnosis: Hypereosinophilia has generally been defined as a peripheral blood eosinophil count greater than 1500/mm(3) and may be associated with tissue damage. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of morphologic review of the blood and marrow, standard cytogenetics, fluorescent in situ-hybridization, flow immunocytometry, and T-cell clonality assessment to detect histopathologic or clonal evidence for an acute or chronic myeloid or lymphoproliferative disorder. Read More

    Prognostic significance of additional chromosomal abnormalities at the time of diagnosis in patients with chronic myeloid leukemia treated with frontline tyrosine kinase inhibitors.
    Am J Hematol 2017 Oct 13. Epub 2017 Oct 13.
    Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.
    Additional cytogenetic abnormalities (ACA) are considered a high risk feature in chronic myeloid leukemia (CML). However, its prognostic significance at the time of diagnosis in the setting of new tyrosine kinase inhibitors (TKIs) is less well understood. Patients with CML in CP with or without ACA at diagnosis treated with frontline TKIs in prospective clinical trials were analyzed for outcomes. Read More

    The athlete's hematological response to hypoxia: A meta-analysis on the influence of altitude exposure on key biomarkers of erythropoiesis.
    Am J Hematol 2017 Oct 13. Epub 2017 Oct 13.
    Aspetar Sports Medicine Hospital, PO Box 29222, Doha, Qatar.
    Altitude training is associated with changes in blood markers, which can confound results of the Athlete?s Biological Passport (ABP). This meta-analysis aims to describe the fluctuations during- and post-altitude in key ABP variables; hemoglobin concentration ([Hb]), square-root transformed reticulocyte percentage (sqrt(retic%)) and the OFF-score. Individual de-identified raw data were provided from 17 studies. Read More

    Therapy related-chronic myelomonocytic leukemia (CMML): Molecular, cytogenetic, and clinical distinctions from de novo CMML.
    Am J Hematol 2017 Oct 11. Epub 2017 Oct 11.
    Division of Hematology, Mayo Clinic, Rochester, Minnesota.
    Therapy related myeloid neoplasms (t-MN) including therapy related myelodysplastic syndromes (t-MDS) and acute myeloid leukemia (t-AML) are associated with aggressive disease biologies and poor outcomes. In this large (n = 497) and informative (inclusive of molecular and cytogenetic information) chronic myelomonocytic leukemia (CMML) patient cohort, we demonstrate key biological insights and an independent prognostic impact for t-CMML. T-CMML was diagnosed in 9% of patients and occurred approximately 7 years after exposure to prior chemotherapy and/or radiation therapy. Read More

    Alternate use of thrombopoietin receptor agonists in adult primary immune thrombocytopenia patients: A retrospective collaborative survey from Italian hematology centers.
    Am J Hematol 2017 Oct 6. Epub 2017 Oct 6.
    Hematology and Oncology Department, Niguarda Cancer Center, ASST Grande Ospedale Metropolitano Niguarda, Milan.
    Sequential use of the TPO-RAs romiplostim and eltrombopag in ITP patients failing either agent was retrospectively evaluated to assess efficacy and impact of clinical characteristics on outcome. Patients were grouped into 5 categories: efficacy issues: 1(st) TPO-RA failure; loss of response; non-efficacy issues: platelet fluctuations; patient's preference; adverse event development. Either one TPO-RA sequence was analyzed at 3 month and at last follow-up. Read More

    Improved prognosis with additional medium-dose VP16 to CY/TBI in allogeneic transplantation for high risk ALL in adults.
    Am J Hematol 2017 Oct 6. Epub 2017 Oct 6.
    Division of Hematology, Jichi Medical University, Saitama, Japan.
    Allogeneic hematopoietic stem cell transplantation (HSCT) with the conventional cyclophosphamide and total body irradiation (CY/TBI) regimen is an essential therapeutic strategy for acute lymphoblastic leukemia (ALL) in adults. Medium-dose etoposide (VP16, 30-40 mg/kg) can be added to intensify this CY/TBI regimen and reduce relapse; however, differences in prognosis between the VP16/CY/TBI and CY/TBI regimens have not yet been fully analyzed. We conducted a retrospective cohort study using a Japanese transplant registry database to compare the prognosis between the VP16/CY/TBI (VP16, total 30-40 mg/kg) (N = 376) and CY/TBI (N = 1178) regimens in adult patients with ALL transplanted at complete remission (CR) between January 1, 2000 and December 31, 2014. Read More

    Hereditary stomatocytosis: An underdiagnosed condition.
    Am J Hematol 2017 Oct 3. Epub 2017 Oct 3.
    Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università degli Studi di Napoli Federico II, Napoli, Italy.
    Hereditary stomatocytoses are a wide class of hemolytic anemias characterized by alterations of ionic flux with increased cation permeability that results in inappropriate shrinkage or swelling of the erythrocytes, and water lost or gained osmotically. The last few years have been crucial for new acquisitions in this field in terms of identifying new causative genes and of studying their pathogenetic mechanisms. This review summarizes the main features of erythrocyte membrane transport diseases, dividing them into forms with either isolated erythroid phenotype (nonsyndromic) or extra-hematological manifestations (syndromic), and focusing particularly on the most recent advances regarding dehydrated forms of hereditary stomatocytosis and familial pseudohyperkalemia. Read More

    Relatively favorable outcome after allogeneic stem cell transplantation for BCR-ABL1-positive AML: A survey from the acute leukemia working party of the European Society for blood and marrow transplantation (EBMT).
    Am J Hematol 2017 Oct 3. Epub 2017 Oct 3.
    Hôpital Saint Antoine, ALWP office, Service d'Hématologie et de Thérapie cellulaire, Paris, France.
    The aim of the study was to assess the role of allogeneic stem cell transplantation (SCT) in patients diagnosed with BCR-ABL1-positive acute myeloid leukemia (AML). Fifty-seven patients (median age, 48 years, range: 19-67) with BCR-ABL1 positive AML undergoing SCT were identified. The majority of the patients (70%) received a TKI before the transplant. Read More

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