9,026 results match your criteria American journal of hematology[Journal]


The effect of azacitidine therapy on the M protein of MDS patients with concomitant MGUS.

Am J Hematol 2018 Jun 15. Epub 2018 Jun 15.

Division of Hematology, Japanese Red Cross Society Wakayama Medical Center, Wakayama, Wakayama, Japan.

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Very poor long-term survival in past and more recent studies for relapsed AML patients: The ECOG-ACRIN experience.

Am J Hematol 2018 Jun 15. Epub 2018 Jun 15.

Memorial Sloan Kettering Cancer, New York, New York.

This study examines the long-term OS of relapsed AML patients who were enrolled to 9 successive ECOG-ACRIN trials for newly diagnosed AML, during 1984-2008. The objectives were to examine whether there is a trend of improvement in the survival of relapsed AML patients in the more recent studies and to search for prognostic factors that are associated with long-term OS after relapse. A total of 3,012 patients were enrolled, 1,779(59. Read More

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Generalization and fine mapping of red blood cell trait genetic associations to multi-ethnic populations: The PAGE Study.

Am J Hematol 2018 Jun 15. Epub 2018 Jun 15.

Department of Epidemiology, University of North Carolina Gillings School of Public Health, Chapel Hill, NC.

Red blood cell (RBC) traits provide insight into a wide range of physiological states and exhibit moderate to high heritability, making them excellent candidates for genetic studies to inform underlying biologic mechanisms. Previous RBC trait genome-wide association studies were performed primarily in European- or Asian-ancestry populations, missing opportunities to inform understanding of RBC genetic architecture in diverse populations and reduce intervals surrounding putative functional SNPs through fine-mapping. Here, we report the first fine-mapping of six correlated (Pearson's r range: |0. Read More

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June 2018
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Red Blood Cell Adhesion to Heme-Activated Endothelial Cells Reflects Clinical Phenotype in Sickle Cell Disease.

Am J Hematol 2018 Jun 15. Epub 2018 Jun 15.

Department of Mechanical and Aerospace Engineering, Case Western Reserve University, Cleveland, OH, USA.

In sickle cell disease (SCD), 'disease severity' associates with increased RBC adhesion to quiescent endothelium, but the impact on activated endothelium is not known. Increased concentrations of free heme result from intravascular hemolysis in SCD. Heme is essential for aerobic metabolism, and plays an important role in numerous biological processes. Read More

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Priapism in sickle cell disease: Beware of neuroleptics.

Am J Hematol 2018 Jun 8. Epub 2018 Jun 8.

Internal medicine department, Sickle Cell Referral Center, Georges Pompidou European Hospital, AP-HP, Paris, France.

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June 2018
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Congenital dyserythropoietic anemia type 1: a case with novel compound heterozygous mutations in the C15orf41 gene.

Am J Hematol 2018 Jun 8. Epub 2018 Jun 8.

Clinical Genetics, Department of Molecular Medicine and Surgery, The Karolinska University Hospital and Karolinska Institutet, Stockholm, Sweden.

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Diagnosis of follicular lymphoma from the peripheral blood.

Authors:
Barbara J Bain

Am J Hematol 2018 Jun 8. Epub 2018 Jun 8.

Department of Haematology, St Mary's Hospital, Praed Street, London W2 1NY, United Kingdom.

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Pancreatic lipomatosis in Diamond-Blackfan anemia: The importance of genetic testing in bone marrow failure disorders.

Am J Hematol 2018 Jun 8. Epub 2018 Jun 8.

Dana-Farber/Boston Children's Cancer and Blood Disorders Center and Harvard Medical School, Boston, MA.

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Shifting Landscape of Hemophilia Therapy: Implications for Current Clinical Laboratory Coagulation Assays.

Am J Hematol 2018 Jun 8. Epub 2018 Jun 8.

Boston Children's Hospital, Boston Hemophilia Center, Boston, MA.

Clinical coagulation assays are an integral part of diagnosing and managing patients with hemophilia; however, in this new era of bioengineered factor products and non-factor therapeutics, problems have arisen with use of traditional coagulation tests for the quantification of several of these new products. Discussion over the use of one-stage clotting assays versus chromogenic substrate assays for clinical decision making and potency labeling has been ongoing for many years. Emerging factor concentrates have heightened concern over assay selection and availability. Read More

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Efficacy of chemotherapy or chemo-anti-PD-1 combination after failed anti-PD-1 therapy for relapsed and refractory Hodgkin lymphoma: A series from Lysa centers.

Am J Hematol 2018 Jun 8. Epub 2018 Jun 8.

Department of Hematology, Centre Hospitalier Lyon Sud, Pierre-Bénite, France.

Anti-PD-1 therapy provides high response rates in Hodgkin lymphoma (HL) patients who have relapsed or are refractory (R/R) to autologous stem cell transplantation (ASCT) and brentuximab vedotin (BV), but median progression free survival (PFS) is only one year. The efficacy of treatment following anti-PD-1 is not well known. We retrospectively investigated the efficacy of salvage therapies for unsatisfactory response to anti-PD-1 therapy, assessed by PET-CT according to the Lugano criteria, in 30 R/R HL patients. Read More

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June 2018
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Chronic myelomonocytic leukemia: 2018 update on diagnosis, risk stratification and management.

Am J Hematol 2018 Jun;93(6):824-840

Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder with overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms, with an inherent risk for leukemic transformation (∼15%-20% over 3-5 years).

Diagnosis: Diagnosis is based on the presence of sustained (>3 months) peripheral blood monocytosis (≥1 × 10 /L; monocytes ≥10%), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in ∼ 30% of patients, while >90% have gene mutations. Read More

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June 2018
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Genotype-Phenotype Correlation of Hereditary Erythrocytosis Mutations, a single center experience.

Am J Hematol 2018 May 23. Epub 2018 May 23.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

Hereditary erythrocytosis is associated with high oxygen affinity hemoglobin variants (HOAs), 2,3-bisphosphoglycerate deficiency and abnormalities in EPOR and the oxygen-sensing pathway proteins PHD, HIF2α, and VHL. Our laboratory has 40 years of experience with hemoglobin disorder testing and we have characterized HOAs using varied protein and molecular techniques including functional assessment by p50 analysis. In addition, we have more recently commenced adding the assessment of clinically relevant regions of the VHL, BPGM, EPOR, EGLN1 (PHD2), and EPAS1 (HIF2A) genes in a more comprehensive hereditary erythrocytosis panel of tests. Read More

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Long-term survival and loss in expectancy of life in a population-based cohort of 7114 patients with diffuse large B-cell lymphoma.

Am J Hematol 2018 May 17. Epub 2018 May 17.

Division of Clinical Epidemiology, Department of Medicin Solna, Karolinska Institutet, Stockholm, Sweden.

Survival has improved among patients with diffuse large B-cell lymphoma (DLBCL) with the addition of anti-CD20 antibody therapy. We aimed to quantify trends and remaining loss in expectation of life (LEL) due to DLBCL at a national population-based level. Patients diagnosed with DLBCL 2000-2013 (N = 7114) were identified through the Swedish Lymphoma Registry and classified according to the age-adjusted International Prognostic Index (aaIPI). Read More

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Exceptional microorganisms on blood smear.

Am J Hematol 2018 May 17. Epub 2018 May 17.

Hematology department, Hôpital Beaujon, HUPNVS, AP-HP, Clichy, France.

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Absence of early HHV-6 reactivation after cord blood allograft predicts powerful graft-versus-tumor effect.

Am J Hematol 2018 May 14. Epub 2018 May 14.

Division of Hematology, Oncology, and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, Minnesota.

Approximately 75% of cord blood transplant (CBT) recipients experience human herpes virus-6 (HHV-6) reactivation. Considering the immunomodulatory effects of HHV-6, we hypothesized that early HHV-6 reactivation may influence the risk of relapse of the underlying hematologic malignancy. In 152 CBT recipients with hematological malignancies, we determined the association between HHV-6 reactivation by day +28 and 2-year cumulative incidence of relapse. Read More

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Did you 'miss' me? A subtle case of intravascular large B-cell lymphoma.

Am J Hematol 2018 May 13. Epub 2018 May 13.

Department of Pathology, Brigham and Women's Hospital, Boston, MA.

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Spotting the elusive Siberian tiger: Complete response to ibrutinib in a patient with Waldenström Macroglobulinemia.

Am J Hematol 2018 May 14. Epub 2018 May 14.

Bing Center for Waldenström Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

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May 2018
2 Reads

Fixed Low-Dose Hydroxyurea for the Treatment of Adults with Sickle Cell Anemia in Nigeria.

Am J Hematol 2018 May 14. Epub 2018 May 14.

Division of Hematology & Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, IL.

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Fertility preservation in women with marrow failure syndromes prior to allogeneic stem cell transplantation.

Am J Hematol 2018 May 13. Epub 2018 May 13.

Department of Obstetrics, Gynecology and Reproductive Biology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

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May 2018
2 Reads

Case series of patients with severe sickle cell disease treated with voxelotor (GBT440) by compassionate access.

Am J Hematol 2018 May 12. Epub 2018 May 12.

Foundation for Sickle Cell Disease Research, Hollywood, Florida.

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rBPI (Opebacan) Promotes Rapid Trilineage Hematopoietic Recovery in a Murine Model of High-Dose Total Body Irradiation.

Am J Hematol 2018 May 11. Epub 2018 May 11.

Department of Radiation Oncology, Dana-Farber Cancer Institute, Boston, MA.

The complexity of providing adequate care after radiation exposure has drawn increasing attention. While most therapeutic development has focused on improving survival at lethal radiation doses, acute hematopoietic syndrome (AHS) occurs at substantially lower exposures. Thus, it is likely that a large proportion of such a radiation-exposed population will manifest AHS of variable degree and that the medical and socioeconomic costs of AHS will accrue. Read More

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Nutritional supplement profile of adults with sickle cell disease.

Am J Hematol 2018 May 4. Epub 2018 May 4.

Division of Hematology, Department of Medicine, Albert Einstein College of Medicine, Bronx, New York.

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Patient-reported health-related quality of life from the phase III TOURMALINE-MM1 study of ixazomib-lenalidomide-dexamethasone versus placebo-lenalidomide-dexamethasone in relapsed/refractory multiple myeloma.

Am J Hematol 2018 May 4. Epub 2018 May 4.

Department of Hematologic Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts.

TOURMALINE-MM1 is a phase III, randomized, double-blind, placebo-controlled study of ixazomib plus lenalidomide and dexamethasone (IRd) versus placebo-Rd in patients with relapsed/refractory multiple myeloma following 1-3 prior lines of therapy. The study met its primary endpoint, demonstrating significantly longer progression-free survival (PFS) in the IRd arm versus placebo-Rd arm (median 20.6 vs 14. Read More

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Laboratory testing in BCR-ABL1-like (Philadelphia-like) B-lymphoblastic leukemia/lymphoma.

Am J Hematol 2018 Apr 26. Epub 2018 Apr 26.

Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts.

BCR-ABL1-like B-lymphoblastic leukemia/lymphoma (BCR-ABL1-like B-ALL), also known as Philadelphia-like (Ph-like) ALL, is a neoplasm of B-lineage lymphoblasts characterized by a pattern of gene expression similar to that of B-ALL with the BCR-ABL1 translocation but lacking the BCR-ABL1 fusion protein. The diagnosis of BCR-ABL1-like B-ALL is associated with a high rate of relapse and poor clinical outcomes. In recognition of the difficulty in screening these leukemias for diagnostic workup, the 2016 update/revision to the World Health Organization (WHO) 2008 edition included BCR-ABL1-like B-ALL as a provisional entity. Read More

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April 2018
2 Reads

Cardiovascular risk factors in immune thrombocytopenia adults: Results from the CARMEN registry.

Am J Hematol 2018 Apr 26. Epub 2018 Apr 26.

Service de Médecine Interne, Institut Universitaire du Cancer de Toulouse-Oncopôle, Toulouse, France.

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Fostamatinib for the treatment of adult persistent and chronic immune thrombocytopenia: Results of two phase 3, randomized, placebo-controlled trials.

Am J Hematol 2018 Apr 26. Epub 2018 Apr 26.

Rigel Pharmaceuticals, South San Francisco, California.

Spleen tyrosine kinase (Syk) signaling is central to phagocytosis-based, antibody-mediated platelet destruction in adults with immune thrombocytopenia (ITP). Fostamatinib, an oral Syk inhibitor, produced sustained on-treatment responses in a phase 2 ITP study. In two parallel, phase 3, multicenter, randomized, double-blind, placebo-controlled trials (FIT1 and FIT2), patients with persistent/chronic ITP were randomized 2:1 to fostamatinib (n = 101) or placebo (n = 49) at 100 mg BID for 24 weeks with a dose increase in nonresponders to 150 mg BID after 4 weeks. Read More

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April 2018
8 Reads

Systematic Detection of Portal or Splenic Vein Thrombosis after Splenectomy for Immune Cytopenia.

Am J Hematol 2018 Apr 20. Epub 2018 Apr 20.

Service de Médecine Interne, Centre de référence des cytopénies auto-immunes de l'adulte, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, Université Paris Est Créteil, Créteil, France.

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April 2018
2 Reads

Plasma proteomics identifies a 'chemokine storm' in idiopathic multicentric Castleman disease.

Am J Hematol 2018 Apr 20. Epub 2018 Apr 20.

University of Pennsylvania, Philadelphia, Pennsylvania.

Human Herpesvirus-8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) is a poorly understood disease involving polyclonal lymphoproliferation with dysmorphic germinal centers, constitutional symptoms, and multi-organ failure. Patients can experience thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly, and normal immunoglobulin levels, - iMCD-TAFRO. Others experience thrombocytosis, milder effusions, and hypergammaglobulinemia, -iMCD-Not Otherwise Specified (iMCD-NOS). Read More

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Angiotensin receptor signaling in sickle cell anemia has a reno-protective effect on urine concentrating ability but results in sickle glomerulopathy.

Am J Hematol 2018 Apr 20. Epub 2018 Apr 20.

Division of Experimental Hematology and Cancer Biology, Cancer and Blood Diseases Institute, Cincinnati Children's Research Foundation, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, 45229.

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April 2018
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Not as sweet as honey: A rare case of an apparent factor V "inhibitor" in association with bee sting anaphylaxis.

Am J Hematol 2018 Apr 20. Epub 2018 Apr 20.

Haematology, Institute of Clinical Pathology and Medical Research (ICPMR), NSW Health Pathology, Westmead Hospital, Westmead, NSW, Australia.

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Outcome of children with newly diagnosed acute lymphoblastic leukemia treated with CCLG-ALL 2008: The first nation-wide prospective multicenter study in China.

Am J Hematol 2018 Apr 20. Epub 2018 Apr 20.

Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center; Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

Acute lymphoblastic leukemia (ALL) is the most common malignancy among children. The trial Chinese Children Leukemia Group (CCLG)-ALL 2008 was a prospective clinical trial designed to improve treatment outcome of childhood ALL through the first nation-wide collaborative study in China. Totally 2231 patients were recruited from ten tertiary hospitals in eight cities. Read More

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April 2018
2 Reads

A case of disseminated Blastic Plasmocytoid Dendritic Cell Neoplasm.

Am J Hematol 2018 Apr 17. Epub 2018 Apr 17.

Hematology Unit, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy.

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April 2018
8 Reads

Time to plateau as a predictor of survival in newly diagnosed multiple myeloma.

Am J Hematol 2018 Apr 16. Epub 2018 Apr 16.

Division of Hematology, Mayo Clinic, Rochester, MN, United States of America.

Response rates in newly diagnosed multiple myeloma have improved dramatically with the introduction of highly effective novel therapies. However, survival in patients achieving optimal responses to initial treatment can vary significantly, and new prognostic indicators are required to improve risk stratification. We investigated the relationship between time to plateau (T ) and survival in 1099 newly diagnosed patients treated with novel agents at our institution from 2005 to 2015. Read More

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The changing face of adult posttransplant lymphoproliferative disorder: Changes in histology between 1999 and 2013.

Am J Hematol 2018 Apr 16. Epub 2018 Apr 16.

Section of Hematology, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut.

Posttransplant lymphoproliferative disorder (PTLD) typically presents with either polymorphic or monomorphic histology. While both are the end result of immunosuppressive therapies, their origins are felt to be different with different prognoses and responsiveness to therapy, resulting in 2 different malignancies. We attempted to confirm reports suggesting that the relative frequency of these 2 histologies is shifting over time. Read More

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Physician decision making in selection of second-line treatments in immune thrombocytopenia in children.

Am J Hematol 2018 Apr 16. Epub 2018 Apr 16.

Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Immune thrombocytopenia (ITP) is an acquired autoimmune bleeding disorder which presents with isolated thrombocytopenia and risk of hemorrhage. While most children with ITP promptly recover with or without drug therapy, ITP is persistent or chronic in others. When needed, how to select second-line therapies is not clear. Read More

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April 2018
3 Reads

The prognostic value of monosomal karyotype (MK) in higher-risk patients with myelodysplastic syndromes treated with 5-Azacitidine: A retrospective analysis of the Hellenic (Greek) Myelodysplastic syndromes Study Group.

Am J Hematol 2018 Apr 16. Epub 2018 Apr 16.

Second Department of Internal Medicine and Research Unit, University General Hospital "Attikon", 1 Rimini St., Haidari, Athens, 12462, Greece.

In this study, we investigated the incidence and prognostic impact of monosomal karyotype (MK) in 405 higher-risk Myelodysplastic Syndromes (MDS) patients treated with 5-AZA. The MK was present in 66 out of 405 (16.3%) patients, most of whom had complex karyotype (CK). Read More

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April 2018
1 Read