46,864 results match your criteria Blood[Journal]


Mechanisms of Action of Ruxolitinib in Murine Models of Hemophagocytic Lymphohistiocytosis.

Blood 2019 Apr 23. Epub 2019 Apr 23.

Oncology, St Jude Children's Research Hospital, United States

Hemophagocytic lymphohistiocytosis (HLH) is an often-fatal disorder characterized by the over-activation of T cells and macrophages that excessively produce pro-inflammatory cytokines, including interferon-γ (IFNγ). Previously, we reported that the Janus kinase (JAK) inhibitor ruxolitinib dampens T cell activation and lessens inflammation in a model of HLH in which perforin-deficient () mice are infected with Lymphocytic Choriomeningitis Virus (LCMV). Ruxolitinib inhibits signaling downstream of IFNγ as well as several other JAK-dependent cytokines. Read More

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http://dx.doi.org/10.1182/blood.2019000761DOI Listing

Clinical and immunological features in a cohort of patients with partial DiGeorge syndrome followed at a single centre.

Blood 2019 Apr 23. Epub 2019 Apr 23.

Great Ormond Street Hospital, Greece.

DiGeorge syndrome (DGS) is a primary immunodeficiency characterized by various degrees of T-cell deficiency. In partial DGS (pDGS), other risk factors could predispose to recurrent infections, autoimmunity and allergy. The aim of this study was to assess the impact of different factors in the development of infections, autoimmunity and/or allergy in patients with pDGS. Read More

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http://dx.doi.org/10.1182/blood.2018885244DOI Listing

Deficiency of adenosine deaminase 2 triggers adenosine-mediated NETosis and TNF production in patients with DADA2.

Blood 2019 Apr 23. Epub 2019 Apr 23.

NIAMS/NIH, United States.

Reduction of adenosine deaminase 2 (ADA2) activity due to autosomal recessive loss-of-function mutations in the ADA2 gene (previously known as CECR1) results in a systemic vasculitis known as deficiency of ADA2 (DADA2). Neutrophils and a subset of neutrophils known as low-density granulocytes (LDGs) have been implicated in the pathogenesis of vasculitis, at least in part, through the formation of neutrophil extracellular traps (NETs). The study objective was to determine whether neutrophils and NETs play a pathogenic role in DADA2. Read More

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http://dx.doi.org/10.1182/blood.2018892752DOI Listing

Reprogramming identifies functionally distinct stages of clonal evolution in myelodysplastic syndromes.

Blood 2019 Apr 22. Epub 2019 Apr 22.

University of Washington, United States

Myeloid neoplasms, including myelodysplastic syndromes (MDS), are genetically heterogeneous disorders driven by clonal acquisition of somatic mutations in hematopoietic stem and progenitor cells (HPCs). The order of premalignant mutations and their impact on HPC self-renewal and differentiation remain poorly understood. We demonstrate that episomal reprogramming of MDS patient samples generates induced pluripotent stem cells (iPSCs) from single premalignant cells with a partial complement of mutations, directly informing the temporal order of mutations in the individual patient. Read More

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http://dx.doi.org/10.1182/blood.2018884338DOI Listing
April 2019
1 Read

Monoclonal gammopathy of undetermined significance (MGUS).

Blood 2019 Apr 22. Epub 2019 Apr 22.

Medical Oncology/Hematologic Malignancies, Dana-Farber Cancer Institute, United States

Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant plasma cell dyscrasia that consistently precedes multiple myeloma (MM) with a 1% risk of progression per year. Recent advances have improved understanding of the complex genetic and immunologic factors that drive and permit progression from aberrant plasma cell clone to MGUS and overt MM. Additional evidence supports a bidirectional interaction of MGUS cells with surrounding cells in the bone marrow niche that regulates malignant transformation. Read More

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http://dx.doi.org/10.1182/blood.2019846782DOI Listing

IGF1R as druggable target mediating PI3K-δ inhibitor resistance in a murine model of chronic lymphocytic leukemia.

Blood 2019 Apr 22. Epub 2019 Apr 22.

Department of Internal Medicine III, University of Ulm, Germany

Targeted therapy is currently revolutionizing the treatment of cancers, but resistance evolves against these therapies and derogates their success. The PI3K-δ inhibitor idelalisib has been approved for CLL and NHL treatment, but the mechanisms conferring resistance in a subset of patients are unknown. Here, we modelled resistance to PI3K-δ inhibitor using a serial tumor transfer and treatment scheme in mice. Read More

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http://dx.doi.org/10.1182/blood.2018881029DOI Listing

Role of urine immunofixation in the complete response assessment of MM patients other than light- chain-only disease.

Blood 2019 Apr 22. Epub 2019 Apr 22.

Hospital Clínic Universitari, IDIBAPS, Barcelona, Spain.

Response criteria for multiple myeloma (MM) require monoclonal protein (M-protein)-negative status on both serum (sIFE) and urine (uIFE) immunofixation-electrophoresis for classification of complete response (CR). However, uIFE is not always performed for sIFE‑negative patients. We analyzed M-protein evaluations from 384 MM patients (excluding those with light-chain-only disease) treated in the GEM2012MENOS65 (NCT01916252) trial in order to determine the uIFE-positive rate in patients who became sIFE-negative post-treatment; and evaluate rates of minimal residual disease (MRD)-negative status and progression-free survival among patients achieving CR, CR but without uIFE available (uncertain CR; uCR), or very good partial response (VGPR). Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood.2019000
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http://dx.doi.org/10.1182/blood.2019000671DOI Listing
April 2019
1 Read

Vitamin K-antagonism impairs the bone marrow microenvironment and hematopoiesis.

Blood 2019 Apr 19. Epub 2019 Apr 19.

Georg-Speyer-Haus, Germany

Vitamin K antagonists (VKA) have been used in 1% of the world's population for prophylaxis or treatment of thromboembolic events for 64 years. Impairment of osteoblast function and osteoporosis has been described in patients on VKA. Given the involvement of cells of the bone marrow microenvironment (BMM), such as mesenchymal stem cells (MSC) and macrophages, as well as other factors like the extracellular matrix for the maintenance of normal hematopoietic stem cells (HSC), we investigated a possible impact of VKA on hematopoiesis via the BMM. Read More

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http://dx.doi.org/10.1182/blood.2018874214DOI Listing

CRISPR/Cas9 PIG-A Gene Editing in Non-Human Primate Model Demonstrates No Intrinsic Clonal Expansion of PNH HSPCs.

Blood 2019 Apr 19. Epub 2019 Apr 19.

Translational Stem Cell Biology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, United States

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http://dx.doi.org/10.1182/blood.2019000800DOI Listing

2018 WHO-EORTC classification update.

Authors:

Blood 2019 Apr;133(16):1791

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http://dx.doi.org/10.1182/blood-2019-03-901066DOI Listing
April 2019
2 Reads

Cannibalistic acute myeloid leukemia with ZMYND11-MBTD1 fusion.

Blood 2019 Apr;133(16):1789

Hospices Civils de Lyon.

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http://dx.doi.org/10.1182/blood-2019-01-898619DOI Listing

αβ changes gears in MKs and platelets.

Blood 2019 Apr;133(16):1700-1701

Kyoto University.

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2019-01
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http://dx.doi.org/10.1182/blood-2019-01-896092DOI Listing
April 2019
1 Read
10.452 Impact Factor

Context-specific tumor suppression by PHF6.

Blood 2019 Apr;133(16):1698-1700

The Jackson Laboratory.

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2019-03
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http://dx.doi.org/10.1182/blood-2019-03-900829DOI Listing
April 2019
1 Read

Storming the Castle with TCP.

Blood 2019 Apr;133(16):1697-1698

University of Arkansas for Medical Sciences.

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2019-02
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http://dx.doi.org/10.1182/blood-2019-02-900803DOI Listing
April 2019
1 Read

Dysfunctional Endogenous FIX Impairs Prophylaxis in a Mouse Hemophilia B Model.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Department of Biology and Pathology, University of North Carolina at Chapel Hill, United States

Factor IX (FIX) binds to collagen IV (Col4) in the sub-endothelial basement membrane. In hemophilia B, this FIX-Col4 interaction reduces the plasma recovery of infused FIX and plays a role in hemostasis. Studies examining the recovery of infused BeneFix (FIX) in null (cross-reactive material negative, CRM) hemophilia B mice suggest the concentration of Col4 readily available for binding FIX is ~405 nM with a 95% confidence interval of 374-436 nM. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood.2018884
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http://dx.doi.org/10.1182/blood.2018884015DOI Listing
April 2019
2 Reads

Inhibition of LSD1 by small molecule inhibitors stimulates fetal hemoglobin synthesis.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Department of Medicine, Section of Hematology & Oncology, Boston University School of Medicine, United States

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http://dx.doi.org/10.1182/blood.2018892737DOI Listing
April 2019
1 Read

Optimizing treatment for HIV-associated lymphoma.

Authors:
Ariela Noy

Blood 2019 Apr 16. Epub 2019 Apr 16.

Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, United States

Cancer is the leading cause of death for HIV-infected persons in economically developed countries, even in the era of antiretroviral therapy (ART). Lymphomas remain a leading cause of cancer morbidity and mortality for HIV-infected patients and have increased incidence even in patients optimally treated with ART. Even limited interruptions of ART can lead to cluster of differentiation 4 (CD4) cell nadirs and HIV viremia, and increase the risk of lymphoma. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-01
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http://dx.doi.org/10.1182/blood-2018-01-791400DOI Listing
April 2019
1 Read

Eltrombopag for refractory severe aplastic anemia: dosing, duration, long term outcomes and clonal evolution.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Translational Stem Cell Biology Branch, National Heart, Lung and Blood Institute, National Institutes of Health, United States

Eltrombopag received FDA approval for the treatment of refractory severe aplastic anemia (rSAA) based on treatment of 43 patients with doses escalating from 50-150mg daily for 12 weeks. Response kinetics suggested that more prolonged administration of EPAG at a dose of 150 mg could speed and improve response rates. We enrolled 40 rSAA patients in a study of EPAG 150mg daily, with a primary endpoint of response at 24 weeks. Read More

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http://dx.doi.org/10.1182/blood.2019000478DOI Listing
April 2019
7 Reads

Engineered Bcor mutations lead to acute leukemia of progenitor B-1 lymphocyte origin in a sensitized background.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Genetics Branch, National Cancer Institute, National Institutes of Health, United States

Approximately 10% of NUP98-PHF23 (NP23) mice develop an aggressive acute lymphoblastic leukemia of B1-lymphocyte progenitor origin (pro B-1 ALL), accompanied by somatic frameshift mutations of the BCL6 interacting corepressor (Bcor) gene, most commonly within a 9 bp "hot spot" in Bcor exon 8. To determine whether experimentally engineered Bcor mutations would lead to pro B-1 ALL, we used CRISPR-Cas9 to introduce a Bcor frameshift mutation into NP23 hematopoietic stem and progenitor cells through the use of Bcor small guide RNAs (Bcor sgRNA). Recipient mice transplanted with NP23 bone marrow (BM) or fetal liver (FL) cells that had been transduced with a Bcor sgRNA developed pro B-1 ALL, characterized by a B-1 progenitor immunophenotype, clonal Igh gene rearrangement, and Bcor indel mutation, whereas control recipients did not. Read More

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http://dx.doi.org/10.1182/blood.2018864173DOI Listing
April 2019
1 Read

Total body irradiation dose and risk of subsequent neoplasms following allogeneic hematopoietic cell transplantation.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Fred Hutchinson Cancer Research Center.

We examined the impact of total body irradiation (TBI) dose and fractionation on risk of subsequent malignant neoplasms (SMN) in the era of reduced intensity and non-myeloablative conditioning regimens for hematopoietic cell transplantation (HCT). Among 4,905 one-year survivors of allogeneic HCT for hematologic malignancies (N=4,500) or non-malignant disorders (N=405) transplanted between 1969-2014 we identified 581 SMNs (excluding squamous and basal cell of skin) in 499 individuals. With a median length of follow-up of 12. Read More

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http://dx.doi.org/10.1182/blood.2018874115DOI Listing

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.

Blood 2019 Apr 16. Epub 2019 Apr 16.

Department of Women's & Children's Health, Karolinska Institutet, Sweden.

Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T-cells. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, while the secondary (acquired) form is most frequent in adults. Secondary HLH is commonly triggered by infections or malignancies but may also be induced by autoinflammatory/autoimmune disorders, in which case it is called macrophage activation syndrome (MAS or MAS-HLH). Read More

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http://dx.doi.org/10.1182/blood.2018894618DOI Listing
April 2019
2 Reads

Immune dysregulation: EBV DLBCL and HLH in a patient with T-LGL.

Blood 2019 Apr;133(15):1695

Icahn School of Medicine at Mount Sinai.

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http://dx.doi.org/10.1182/blood-2019-01-899484DOI Listing

Congenital TTP: toward a turning point.

Authors:
James N George

Blood 2019 Apr;133(15):1615-1617

University of Oklahoma Health Sciences Center.

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http://dx.doi.org/10.1182/blood-2019-02-900795DOI Listing

I myelofibrosis! Veni VitD! Et tu, macrophage?

Authors:
Ashley P Ng

Blood 2019 Apr;133(15):1613-1615

The Walter and Eliza Hall Institute of Medical Research.

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2019-02
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http://dx.doi.org/10.1182/blood-2019-02-900779DOI Listing
April 2019
2 Reads

CRISPR/Cas9-mediated in vivo gene targeting corrects hemostasis in newborn and adult factor IX knockout mice.

Blood 2019 Apr 11. Epub 2019 Apr 11.

Department of Medicine, University of Pennsylvania, United States

Many genetic diseases, including hemophilia, require long-term therapeutic effects. Despite the initial success of liver-directed adeno-associated virus (AAV) gene therapy for hemophilia in clinical trials, long-term, sustained therapeutic effects have yet to be seen. One explanation of the gradual decline of efficacy over time is that the non-integrating AAV vector genome could be lost during cell division during hepatocyte turnover, albeit at a slow pace in adults. Read More

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http://dx.doi.org/10.1182/blood.2019000790DOI Listing
April 2019
1 Read

PD-1/PD-L1 immune checkpoint and p53 loss facilitate tumor progression in activated B cell diffuse large B-cell lymphomas.

Blood 2019 Apr 11. Epub 2019 Apr 11.

Hemato-Oncology, Center for Applied Medical Research (CIMA) University of Navarra, Spain

Refractory or relapsed diffuse large B-cell lymphoma (DLBCL) often associates with the activated B cell-like (ABC) subtype and genetic alterations that drive constitutive NF-kB activation and impair B cell terminal differentiation. Here, we show that DNA damage response by p53 is a central mechanism suppressing the pathogenic cooperation of IKK2ca-enforced canonical NF-kB and impaired differentiation due to Blimp1 loss in ABC-DLBCL lymphomagenesis. We provide evidences that the interplay between these genetic alterations and the tumor microenvironment (TME) select for additional molecular addictions that promote lymphoma progression, including aberrant co-expression of FOXP1 and the B-cell mutagenic enzyme AID, and immune evasion through MHC-II downregulation, PD-L1 upregulation and T-cell exhaustion. Read More

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http://dx.doi.org/10.1182/blood.2018889931DOI Listing
April 2019
1 Read
10.452 Impact Factor

The heparin binding domain of von Willebrand factor binds to growth factors and promotes angiogenesis in wound healing.

Blood 2019 Apr 11. Epub 2019 Apr 11.

Institute for Molecular Engineering, University of Chicago, United States

During wound healing, the distribution, availability and signaling of growth factors (GFs) are orchestrated by their binding to extracellular matrix components in the wound microenvironment. Extracellular matrix proteins have been shown to modulate angiogenesis and promote wound healing through GFs binding. The hemostatic protein von Willebrand factor (VWF), released by endothelial cells (ECs) in plasma and in the subendothelial matrix, has been shown to regulate angiogenesis; this function is relevant to patients where VWF deficiency or dysfunction is associated with vascular malformations. Read More

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http://dx.doi.org/10.1182/blood.2019000510DOI Listing
April 2019
1 Read

Regulation of gene expression by miR-144/451 during mouse erythropoiesis.

Blood 2019 Apr 10. Epub 2019 Apr 10.

Division of Hematology-Oncology, University of Pennsylvania, United States.

The microRNA (miRNA) locus miR-144/451 is abundantly expressed in erythrocyte precursors, facilitates their terminal maturation and protects against oxidant stress. However, the full repertoire of erythroid miR-144/451 target mRNAs and associated cellular pathways is unknown. In general, the numbers of mRNAs predicted to be targeted by a miRNA vary greatly from hundreds to thousands, and are dependent on experimental approaches. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood.2018854
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http://dx.doi.org/10.1182/blood.2018854604DOI Listing
April 2019
1 Read

RNA binding proteins in hematopoiesis and hematological malignancy.

Blood 2019 Apr 9. Epub 2019 Apr 9.

Wellcome MRC Stem Cell Institute & Department of Haematology, University of Cambridge, Cambridge, United Kingdom;

RNA binding proteins (RBPs) regulate fundamental processes such as differentiation and self-renewal by enabling the dynamic control of protein abundance or isoforms, or through the regulation of non-coding RNA. RBPs are increasingly appreciated as being essential for normal hematopoiesis and they are understood to play fundamental roles in hematological malignancies by acting as oncogenes or tumor suppressors. Alternative splicing has been shown to play roles in the development of specific hematopoietic lineages and sequence specific mutations in RBPs lead to dysregulated splicing in myeloid and lymphoid leukemias. Read More

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http://dx.doi.org/10.1182/blood-2018-10-839985DOI Listing

Acalabrutinib for Mantle Cell Lymphoma.

Blood 2019 Apr 9. Epub 2019 Apr 9.

Division of Hematology, Mayo Clinic, United States.

Mantle cell lymphoma (MCL) is a unique type of non-Hodgkin lymphoma (NHL) characterized by the overexpression of cyclin D1. MCL patients typically live years but experience multiple relapses. Acalabrutinib (Acala) is a novel second generation oral Bruton's tyrosine kinase (BTK) inhibitor approved by the US FDA for relapsed MCL based on a clinical trial demonstrating an overall response rate of 81%. Read More

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http://dx.doi.org/10.1182/blood.2019852368DOI Listing
April 2019
3 Reads

Proteomics evidence of specific IGKV1-8 association with cystic lung light chain deposition disease.

Blood 2019 Apr 9. Epub 2019 Apr 9.

pathology, Institut Universitaire du Cancer (IUCT), France

We previously reported a new form of LCDD presenting as diffuse cystic lung disorder that differs from the usual systemic form, with respect to the age, the male/female ratio, the involved organs, and the hematologic characteristics. We also demonstrated that the light chains were produced by an intrapulmonary B-cell clone and, that this clone shared a stereotyped antigen receptor IGHV4-34/IGKV1. However, we analyzed only 3 patients. Read More

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http://dx.doi.org/10.1182/blood.2019898577DOI Listing
April 2019
2 Reads

Chronic CD30-signaling in B cells results in lymphomagenesis by driving the expansion of plasmablasts and B1 cells.

Blood 2019 Apr 8. Epub 2019 Apr 8.

Research Unit Gene Vectors, Helmholtz Zentrum München - German Research Center for Environmental Health (GmbH), Germany

CD30 is expressed on a variety of B-cell lymphomas such as Hodgkin's lymphoma, primary effusion lymphoma, and a subgroup of diffuse large B-cell lymphoma. In normal tissues, CD30 is expressed on some activated B and T lymphocytes. However, the physiological function of CD30-signaling and its contribution to the generation of CD30-positive lymphomas are still poorly understood. Read More

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http://dx.doi.org/10.1182/blood.2018880138DOI Listing
April 2019
8 Reads

Red blood cells modulate structure and dynamics of venous clot formation in sickle cell disease.

Blood 2019 Apr 5. Epub 2019 Apr 5.

University of North Carolina at Chapel Hill, United States

Sickle cell disease (SCD) is associated with chronic activation of coagulation and an increased risk of venous thromboembolism (VTE). Erythrocyte sickling, the primary pathologic event in SCD, results in dramatic morphological changes in RBCs due to polymerization of the abnormal hemoglobin. We used a mouse model of SCD and blood samples from sickle patients to determine if these changes affect the structure, properties and dynamics of sickle clot formation. Read More

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http://dx.doi.org/10.1182/blood.2019000424DOI Listing

Inhibition of platelet GPVI induces intratumor hemorrhage and increases efficacy of chemotherapy in mice.

Blood 2019 Apr 5. Epub 2019 Apr 5.

Institute of Experimental Biomedicine, University of Würzburg, Germany

Maintenance of tumor vasculature integrity is indispensable for tumor growth and thus affects tumor progression. Previous studies have identified platelets as major regulators of tumor vascular integrity as their depletion selectively rendered tumor vessels highly permeable and caused massive intratumoral hemorrhage. While these results established platelets as potential targets for anti-tumor therapy, their depletion is not a treatment option due to their essential role for hemostasis. Read More

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http://dx.doi.org/10.1182/blood.2018877043DOI Listing

PD-1 Blockade with Pembrolizumab for Classical Hodgkin Lymphoma after Autologous Stem Cell Transplantation.

Blood 2019 Apr 5. Epub 2019 Apr 5.

City of Hope Medical Center, United States.

Autologous stem cell transplantation (ASCT) remains the standard of care for patients with relapsed/refractory classical Hodgkin lymphoma (RR cHL) who respond to salvage chemotherapy. However, relapse after ASCT remains a frequent cause of treatment failure, with poor subsequent prognosis. Since cHL is uniquely vulnerable to PD-1 blockade, PD-1 blockade given as consolidation after ASCT could improve ASCT outcomes. Read More

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http://dx.doi.org/10.1182/blood.2019000215DOI Listing
April 2019
3 Reads

Identification of a novel enhancer of CEBPE essential for granulocytic differentiation.

Blood 2019 Apr 5. Epub 2019 Apr 5.

Cancer Science Institute of Singapore.

CCAAT/enhancer binding protein epsilon (CEBPE) is an essential transcription factor for granulocytic differentiation. Mutations of CEBPE occur in individuals with neutrophil-specific granule deficiency (SGD), which is characterized by defects in neutrophil maturation. Cebpe knockout mice also exhibit defects in terminal differentiation of granulocytes, a phenotype reminiscent of SGD. Read More

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http://dx.doi.org/10.1182/blood.2018886077DOI Listing
April 2019
1 Read

Asrij/OCIAD1 suppresses CSN5-mediated p53 degradation and maintains mouse hematopoietic stem cell quiescence.

Blood 2019 Apr 5. Epub 2019 Apr 5.

Molecular Biology and Genetics Unit, Jawaharlal Nehru Centre for Advanced Scientific Research, India

Inactivation of the tumor suppressor p53 is essential for unrestrained growth of cancers. However, only 11% of hematological malignancies have mutant p53. Mechanisms that cause wild type p53 dysfunction and promote leukemia are inadequately deciphered. Read More

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http://dx.doi.org/10.1182/blood.2019000530DOI Listing
April 2019
6 Reads

Contamination.

Blood 2019 Apr;133(14):1612

Centre Hospitalier Universitaire Vaudois.

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http://dx.doi.org/10.1182/blood-2019-01-894451DOI Listing
April 2019
1 Read

Biphenotypic plasma cell myeloma.

Blood 2019 Apr;133(14):1611

Brown University.

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http://dx.doi.org/10.1182/blood-2019-01-898171DOI Listing

Ponatinib and platelets a conflict in CML.

Blood 2019 Apr;133(14):1520-1521

INSERM Clinical Investigation Centre 1402.

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http://dx.doi.org/10.1182/blood-2019-02-900472DOI Listing
April 2019
1 Read

Pediatric Evans syndrome is associated with a high frequency of potentially damaging variants in immune genes.

Blood 2019 Apr 2. Epub 2019 Apr 2.

Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, INSERM UMR 1163-Institut Imagine, Paris, France.

Evans syndrome (ES) is a rare severe autoimmune disorder characterized by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. In most cases, the underlying cause is unknown. We sought to identify genetic defects in pediatric ES (pES), based on a hypothesis of strong genetic determinism. Read More

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http://dx.doi.org/10.1182/blood-2018-11-887141DOI Listing
April 2019
1 Read

Prognostic impact of circulating tumor DNA status post-allogeneic hematopoietic stem cell transplantation in AML and MDS.

Blood 2019 Apr 1. Epub 2019 Apr 1.

Department of Hematology/Oncology, Research Hospital, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan;

This study was performed to assess the utility of tumor-derived fragmentary DNA, or circulating tumor DNA (ctDNA), for identifying high-risk patients for relapse of acute myeloid leukemia and myelodysplastic syndrome (AML/MDS) after undergoing myeloablative allogeneic stem cell transplantation (alloSCT). We retrospectively collected tumor and available matched serum samples at diagnosis and 1 and 3 months post-alloSCT from 53 patients with AML/MDS. After identifying driver mutations in 51 patients using next-generation sequencing, we designed at least one personalized digital-PCR assay per case. Read More

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http://dx.doi.org/10.1182/blood-2018-10-880690DOI Listing
April 2019
4 Reads

ERCC6L2 defines a novel entity within inherited acute myeloid leukemia.

Blood 2019 Apr 1. Epub 2019 Apr 1.

Department of Hematology, Helsinki University Hospital Comprehensive Cancer Center and University of Helsinki, Helsinki, Finland.

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http://dx.doi.org/10.1182/blood-2019-01-896233DOI Listing

How I manage anticoagulant therapy in older individuals with atrial fibrillation or venous thromboembolism.

Blood 2019 Mar 29. Epub 2019 Mar 29.

Department of Medicine, McMaster University, Hamilton, ON, Canada.

Anticoagulant therapy is the most effective strategy to prevent arterial and venous thromboembolism but treating older individuals is challenging because increasing age, comorbidities and polypharmacy increase the risk of both thrombosis and bleeding. Warfarin and the non-vitamin K antagonist oral anticoagulants are underused and often under-dosed in the prevention of stroke in older patients with atrial fibrillation because of concerns about the risk of bleeding. Poor adherence to anticoagulant therapy is also an issue for older patients with atrial fibrillation and for those at risk of recurrent pulmonary embolism. Read More

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http://dx.doi.org/10.1182/blood-2019-01-846048DOI Listing
March 2019
1 Read