Cord blood transplantation and refractory severe anaplastic anemia
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Tags: Free Research Articles, CME article CONTINUING MEDICAL EDUCATION (CME) QUESTIONS Source Type: research

Exflagellation of Plasmodium vivax microgametocytes in human peripheral blood
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Singh Bhar, V., Agarwal, M. Tags: Free Research Articles, BloodWork, Red Cells, Iron, and Erythropoiesis BLOOD WORK Source Type: research

Harlequin cells
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Hirano, T., Eto, K. Tags: Free Research Articles, BloodWork, Myeloid Neoplasia BLOOD WORK Source Type: research

Primary cutaneous small/medium CD4+ T-cell lymphoproliferative disorder
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Xu, J., Huang, Q. Tags: Free Research Articles, BloodWork, Lymphoid Neoplasia BLOOD WORK Source Type: research

Treatment of IgM-associated immunoglobulin light-chain amyloidosis with rituximab-bendamustine
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Manwani, R., Sachchithanantham, S., Mahmood, S., Foard, D., Sharpley, F., Rezk, T., Lane, T., Quarta, C., Fontana, M., Lachmann, H. J., Gillmore, J. D., Whelan, C., Hawkins, P. N., Wechalekar, A. D. Tags: Multiple Myeloma, Lymphoid Neoplasia, Clinical Trials and Observations Letter to Blood Source Type: research

CCR4 mutations associated with superior outcome of adult T-cell leukemia/lymphoma under mogamulizumab treatment
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Sakamoto, Y., Ishida, T., Masaki, A., Murase, T., Yonekura, K., Tashiro, Y., Tokunaga, M., Utsunomiya, A., Ito, A., Kusumoto, S., Iida, S., Ueda, R., Inagaki, H. Tags: Immunobiology and Immunotherapy, Lymphoid Neoplasia, Clinical Trials and Observations Letter to Blood Source Type: research

Heparin-induced thrombocytopenia and thrombosis during high dose melphalan and autologous stem cell transplantation
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Sarosiek, S., Quillen, K., Sloan, J. M., Brauneis, D., Sanchorawala, V. Tags: Thrombocytopenia, Multiple Myeloma, Platelets and Thrombopoiesis, Thrombosis and Hemostasis, Clinical Trials and Observations Letter to Blood Source Type: research

Unrelated cord blood transplantation in patients with idiopathic refractory severe aplastic anemia: a nationwide phase 2 study
Outcomes remain poor for refractory severe aplastic anemia (SAA) patients. Alternative donor transplantation may be considered, but results from previous studies are not encouraging. We conducted a prospective nationwide phase 2 study to assess unrelated cord blood (CB) transplantation (CBT) efficacy and safety in refractory SAA patients (Aplastic Anemia and Cord Blood Transplantation protocol). To demonstrate a significant difference in 1-year survival from 20% (null hypothesis) to 50% (alternative hypothesis), we needed to include 25 transplanted patients and therefore included 26 (median age, 16 years). Eligibility crit...
Source: Blood - August 16, 2018 Category: Hematology Authors: Peffault de Latour, R., Chevret, S., Jubert, C., Sirvent, A., Galambrun, C., Ruggeri, A., Gandemer, V., Cornillon, J., Rialland, F., Dalle, J.-H., Forcade, E., Bruno, B., Paillard, C., Rorlich, P. S., Salmon, A., Fürst, S., Sicre de Fontbrune, F., Tags: Pediatric Hematology, Transplantation, Free Research Articles, Red Cells, Iron, and Erythropoiesis, CME article, Brief Reports, Clinical Trials and Observations Source Type: research

Self-repopulating recipient bone marrow resident macrophages promote long-term hematopoietic stem cell engraftment
Distinct subsets of resident tissue macrophages are important in hematopoietic stem cell niche homeostasis and erythropoiesis. We used a myeloid reporter gene (Csf1r-eGFP) to dissect the persistence of bone marrow and splenic macrophage subsets following lethal irradiation and autologous hematopoietic stem cell transplantation in a mouse model. Multiple recipient bone marrow and splenic macrophage subsets survived after autologous hematopoietic stem cell transplantation with organ-specific persistence kinetics. Short-term persistence (5 weeks) of recipient resident macrophages in spleen paralleled the duration of extramedu...
Source: Blood - August 16, 2018 Category: Hematology Authors: Kaur, S., Raggatt, L. J., Millard, S. M., Wu, A. C., Batoon, L., Jacobsen, R. N., Winkler, I. G., MacDonald, K. P., Perkins, A. C., Hume, D. A., Levesque, J.-P., Pettit, A. R. Tags: Hematopoiesis and Stem Cells, Transplantation Source Type: research

Dynamic intercellular redistribution of HIT antigen modulates heparin-induced thrombocytopenia
Heparin-induced thrombocytopenia (HIT) is a prothrombotic disorder initiated by antibodies to platelet factor 4 (PF4)/heparin complexes. PF4 released from platelets binds to surface glycosaminoglycans on hematopoietic and vascular cells that are heterogenous in composition and differ in affinity for PF4. PF4 binds to monocytes with higher affinity than to platelets, and depletion of monocytes exacerbates thrombocytopenia in a murine HIT model. Here we show that the expression of PF4 on platelets and development of thrombocytopenia are modulated by the (re)distribution of PF4 among hematopoietic and endothelial cell surface...
Source: Blood - August 16, 2018 Category: Hematology Authors: Dai, J., Madeeva, D., Hayes, V., Ahn, H. S., Tutwiler, V., Arepally, G. M., Cines, D. B., Poncz, M., Rauova, L. Tags: Thrombocytopenia, Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research

Transfusion dependence, use of hospice services, and quality of end-of-life care in leukemia
We examined the association between TD and end-of-life outcomes among Medicare beneficiaries with leukemia. From the Surveillance, Epidemiology, and End Results-Medicare database, we selected beneficiaries with acute and chronic leukemias who died in 2001-2011. We defined TD as ≥2 transfusions within 30 days before death or hospice enrollment. End points included hospice enrollment and length of stay, reporting relative risk (RR) adjusted for key covariates. Among 21 033 patients with a median age of 79 years, 20% were transfusion dependent before death/hospice enrollment. Use of hospice increased from 35% in 2001 to 49...
Source: Blood - August 16, 2018 Category: Hematology Authors: LeBlanc, T. W., Egan, P. C., Olszewski, A. J. Tags: Transfusion Medicine, Myeloid Neoplasia, Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research

Cancer-associated thrombosis in patients with implanted ports: a prospective multicenter French cohort study (ONCOCIP)
In conclusion, this large prospective cohort study showed a high rate of venous thromboembolism in patients with a solid tumor and implanted port. Risk factors for catheter-related thrombosis differed from those for venous thromboembolism not catheter-related. This trial was registered at www.clinicaltrials.gov as #NCT02025894. (Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Decousus, H., Bourmaud, A., Fournel, P., Bertoletti, L., Labruyere, C., Presles, E., Merah, A., Laporte, S., Stefani, L., Piano, F. D., Jacquin, J.-P., Meyer, G., Chauvin, F., for the ONCOCIP Investigators Tags: Thrombosis and Hemostasis, Clinical Trials and Observations Source Type: research

Aggressive B-cell lymphomas in patients with myelofibrosis receiving JAK1/2 inhibitor therapy
Inhibition of Janus-kinase 1/2 (JAK1/2) is a mainstay to treat myeloproliferative neoplasms (MPN). Sporadic observations reported the co-incidence of B-cell non-Hodgkin lymphomas during treatment of MPN with JAK1/2 inhibitors. We assessed 626 patients with MPN, including 69 with myelofibrosis receiving JAK1/2 inhibitors for lymphoma development. B-cell lymphomas evolved in 4 (5.8%) of 69 patients receiving JAK1/2 inhibition compared with 2 (0.36%) of 557 with conventional treatment (16-fold increased risk). A similar 15-fold increase was observed in an independent cohort of 929 patients with MPN. Considering primary myelof...
Source: Blood - August 16, 2018 Category: Hematology Authors: Porpaczy, E., Tripolt, S., Hoelbl-Kovacic, A., Gisslinger, B., Bago-Horvath, Z., Casanova-Hevia, E., Clappier, E., Decker, T., Fajmann, S., Fux, D. A., Greiner, G., Gueltekin, S., Heller, G., Herkner, H., Hoermann, G., Kiladjian, J.-J., Kolbe, T., Kornaut Tags: Free Research Articles, Myeloid Neoplasia, Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research

L-Glutamine for sickle cell anemia: more questions than answers
In 2017, the Food and Drug Administration approved 2 medications for sickle cell anemia (SCA): hydroxyurea for children and l-glutamine for children and adults. The approval of hydroxyurea was long overdue, but the approval of l-glutamine was a surprise to many. Any effective new treatment for SCA is a welcome advance, but there are few published studies of l-glutamine as a specific treatment for SCA. Accordingly, there are many unanswered questions about its efficacy, safety, and role in current therapy. (Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Quinn, C. T. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis, Blood Spotlight, Clinical Trials and Observations Source Type: research

Novel agents for primary central nervous system lymphoma: evidence and perspectives
Primary central nervous system lymphoma (PCNSL) is a rare aggressive extranodal non- Hodgkin lymphoma. Although high remission rates can be achieved with high-dose methotrexate–based immunochemotherapy, risk of relapse and associated death is still substantial in at least a third of patients. Novel agents for treating lymphoid malignancies have substantially enriched treatment options for PCNSL. We herein systematically review the existing clinical evidence of novel agents in treatment of PCNSL, summarize ongoing studies, and discuss perspectives. The body of evidence for novel agents is still limited to noncomparati...
Source: Blood - August 16, 2018 Category: Hematology Authors: Illerhaus, G., Schorb, E., Kasenda, B. Tags: Perspectives, Immunobiology and Immunotherapy, Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research

Moving target PF4 directs HIT responses
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Semple, J. W., Kapur, R. Tags: Free Research Articles COMMENTS Source Type: research

Non-zero-sum game of transfusions: EOL in leukemia
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Medeiros, B. C. Tags: Free Research Articles COMMENTS Source Type: research

Benefits and risks of JAK inhibition
(Source: Blood)
Source: Blood - August 16, 2018 Category: Hematology Authors: Arcaini, L., Cazzola, M. Tags: Free Research Articles COMMENTS Source Type: research

Extracellular vesicles in DLBCL provide abundant clues to aberrant transcriptional programming and genomic alterations
The biological role of extracellular vesicles (EVs) in diffuse large B-cell lymphoma (DLBCL) initiation and progression remains largely unknown. We characterized EVs secreted by 5 DLBCL cell lines, a primary DLBCL tumor, and a normal control B-cell sample, optimized their purification, and analyzed their content. We found that DLBCLs secreted large quantities of CD63, Alix, TSG101, and CD81 EVs, which can be extracted using an ultracentrifugation-based method and traced by their cell of origin surface markers. We also showed that tumor-derived EVs can be exchanged between lymphoma cells, normal tonsillar cells, and HK stro...
Source: Blood - August 16, 2018 Category: Hematology Authors: Rutherford, S. C., Fachel, A. A., Li, S., Sawh, S., Muley, A., Ishii, J., Saxena, A., Dominguez, P. M., Caldas Lopes, E., Agirre, X., Chambwe, N., Correa, F., Jiang, Y., Richards, K. L., Betel, D., Shaknovich, R. Tags: Lymphoid Neoplasia, e-Blood Source Type: research

Rasburicase-induced hemolytic anemia in previously undiagnosed G6PD deficiency
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Ferguson, D., Kovach, A. E. Tags: Transfusion Medicine, Free Research Articles, BloodWork, Red Cells, Iron, and Erythropoiesis BLOOD WORK Source Type: research

A universal solution for eliminating false positives in myeloma due to therapeutic monoclonal antibody interference
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Mills, J. R., Kohlhagen, M. C., Willrich, M. A. V., Kourelis, T., Dispenzieri, A., Murray, D. L. Tags: Multiple Myeloma, Lymphoid Neoplasia Letter to Blood Source Type: research

Rituximab monotherapy in splenic marginal zone lymphoma: prolonged responses and potential benefit from maintenance
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Kalpadakis, C., Pangalis, G. A., Sachanas, S., Tsirkinidis, P., Kontopidou, F. N., Moschogiannis, M., Yiakoumis, X., Koulieris, E., Dimopoulou, M. N., Kokkoris, S. I., Kyrtsonis, M.-C., Siakantaris, M. P., Tsourouflis, G., Korkolopoulou, P., Rontogianni, Tags: Lymphoid Neoplasia Letter to Blood Source Type: research

Improved CNS exposure to tocilizumab after cerebrospinal fluid compared to intravenous administration in rhesus macaques
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Nellan, A., McCully, C. M. L., Cruz Garcia, R., Jayaprakash, N., Widemann, B. C., Lee, D. W., Warren, K. E. Tags: Immunobiology and Immunotherapy Letter to Blood Source Type: research

Syndromic congenital myelofibrosis associated with a loss-of-function variant in RBSN
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Magoulas, P. L., Shchelochkov, O. A., Bainbridge, M. N., Ben-Shachar, S., Yatsenko, S., Potocki, L., Lewis, R. A., Searby, C., Marcogliese, A. N., Elghetany, M. T., Zapata, G., Hernandez, P. P., Gadkari, M., Einhaus, D., Muzny, D. M., Gibbs, R. A., Bertuc Tags: Hematopoiesis and Stem Cells, Phagocytes, Granulocytes, and Myelopoiesis, Clinical Trials and Observations Letter to Blood Source Type: research

Warfarin and vitamin K epoxide reductase: a molecular accounting for observed inhibition
Vitamin K epoxide reductase (VKOR), an endoplasmic reticulum membrane protein, is the key enzyme for vitamin K–dependent carboxylation, a posttranslational modification that is essential for the biological functions of coagulation factors. VKOR is the target of the most widely prescribed oral anticoagulant, warfarin. However, the topological structure of VKOR and the mechanism of warfarin’s inhibition of VKOR remain elusive. Additionally, it is not clear why warfarin-resistant VKOR mutations identified in patients significantly decrease warfarin’s binding affinity, but have only a minor effect on vitamin ...
Source: Blood - August 9, 2018 Category: Hematology Authors: Wu, S., Chen, X., Jin, D.-Y., Stafford, D. W., Pedersen, L. G., Tie, J.-K. Tags: Thrombosis and Hemostasis Source Type: research

ADAP deficiency impairs megakaryocyte polarization with ectopic proplatelet release and causes microthrombocytopenia
In this study, we used constitutive ADAP-deficient mice (Adap–/–) as a model to investigate mechanisms underlying the microthrombocytopenia in CARST. We show that Adap–/– mice display several characteristics of human CARST, with moderate thrombocytopenia and smaller-sized platelets. Adap–/– platelets had a shorter life span than control platelets, and macrophage depletion, but not splenectomy, increased platelet counts in mutant mice to control levels. Whole-sternum 3-dimensional confocal imaging and intravital 2-photon microscopy revealed altered morphology of ADAP-deficient MKs with si...
Source: Blood - August 9, 2018 Category: Hematology Authors: Spindler, M., van Eeuwijk, J. M. M., Schurr, Y., Nurden, P., Nieswandt, B., Stegner, D., Reinhold, A., Bender, M. Tags: Thrombocytopenia, Platelets and Thrombopoiesis Source Type: research

GPIb{alpha} is required for platelet-mediated hepatic thrombopoietin generation
Thrombopoietin (TPO), a hematopoietic growth factor produced predominantly by the liver, is essential for thrombopoiesis. Prevailing theory posits that circulating TPO levels are maintained through its clearance by platelets and megakaryocytes via surface c-Mpl receptor internalization. Interestingly, we found a two- to threefold decrease in circulating TPO in GPIbα–/– mice compared with wild-type (WT) controls, which was consistent in GPIbα-deficient human Bernard-Soulier syndrome (BSS) patients. We showed that lower TPO levels in GPIbα-deficient conditions were not due to increased TPO clear...
Source: Blood - August 9, 2018 Category: Hematology Authors: Xu, M., Li, J., Neves, M. A. D., Zhu, G., Carrim, N., Yu, R., Gupta, S., Marshall, J., Rotstein, O., Peng, J., Hou, M., Kunishima, S., Ware, J., Branch, D. R., Lazarus, A. H., Ruggeri, Z. M., Freedman, J., Ni, H. Tags: Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research

Srsf2P95H initiates myeloid bias and myelodysplastic/myeloproliferative syndrome from hemopoietic stem cells
We describe a new conditional murine Srsf2P95H mutation model, where the P95H mutation is expressed physiologically and heterozygously from its endogenous locus after Cre activation. Using multiple Cre lines, we demonstrate that during native hemopoiesis (ie, no BM transplantation), the Srsf2P95H mutation needs to occur within the hemopoietic stem-cell–containing populations to promote myelomonocytic bias and expansion with corresponding transcriptional and RNA splicing changes. With age, nontransplanted Srsf2P95H animals developed a progressive, transplantable disease characterized by myeloid bias, morphological dys...
Source: Blood - August 9, 2018 Category: Hematology Authors: Smeets, M. F., Tan, S. Y., Xu, J. J., Anande, G., Unnikrishnan, A., Chalk, A. M., Taylor, S. R., Pimanda, J. E., Wall, M., Purton, L. E., Walkley, C. R. Tags: Hematopoiesis and Stem Cells, Myeloid Neoplasia Source Type: research

Phase 2b study of 2 dosing regimens of quizartinib monotherapy in FLT3-ITD-mutated, relapsed or refractory AML
This randomized, open-label, phase 2b study (NCT01565668) evaluated the efficacy and safety of 2 dosing regimens of quizartinib monotherapy in patients with relapsed/refractory (R/R) FLT3-internal tandem duplication (ITD)–mutated acute myeloid leukemia (AML) who previously underwent transplant or 1 second-line salvage therapy. Patients (N = 76) were randomly assigned to 30- or 60-mg/day doses (escalations to 60 or 90 mg/day, respectively, permitted for lack/loss of response) of single-agent oral quizartinib dihydrochloride. Allelic frequency of at least 10% was defined as FLT3-ITD–mutated disease. Coprimary end...
Source: Blood - August 9, 2018 Category: Hematology Authors: Cortes, J. E., Tallman, M. S., Schiller, G. J., Trone, D., Gammon, G., Goldberg, S. L., Perl, A. E., Marie, J.-P., Martinelli, G., Kantarjian, H. M., Levis, M. J. Tags: Myeloid Neoplasia, Clinical Trials and Observations Source Type: research

Identification of novel mutational drivers reveals oncogene dependencies in multiple myeloma
Understanding the profile of oncogene and tumor suppressor gene mutations with their interactions and impact on the prognosis of multiple myeloma (MM) can improve the definition of disease subsets and identify pathways important in disease pathobiology. Using integrated genomics of 1273 newly diagnosed patients with MM, we identified 63 driver genes, some of which are novel, including IDH1, IDH2, HUWE1, KLHL6, and PTPN11. Oncogene mutations are significantly more clonal than tumor suppressor mutations, indicating they may exert a bigger selective pressure. Patients with more driver gene abnormalities are associated with wo...
Source: Blood - August 9, 2018 Category: Hematology Authors: Walker, B. A., Mavrommatis, K., Wardell, C. P., Ashby, T. C., Bauer, M., Davies, F. E., Rosenthal, A., Wang, H., Qu, P., Hoering, A., Samur, M., Towfic, F., Ortiz, M., Flynt, E., Yu, Z., Yang, Z., Rozelle, D., Obenauer, J., Trotter, M., Auclair, D., Keats Tags: Lymphoid Neoplasia Source Type: research

CK1{alpha} and IRF4 are essential and independent effectors of immunomodulatory drugs in primary effusion lymphoma
In conclusion, IMiD toxicity in PEL cell lines is independent of IKZF1 and IKZF3 but proceeds through degradation of the neosubstrate CK1α and downregulation of IRF4. (Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Patil, A., Manzano, M., Gottwein, E. Tags: Immunobiology and Immunotherapy, Lymphoid Neoplasia Source Type: research

Aged murine hematopoietic stem cells drive aging-associated immune remodeling
We report that both phenotypic and functional changes in the immune system on aging are primarily a consequence of changes in the function of HSCs on aging and, to a large extent, independent of the thymus, as young and aged HSCs reconstituted distinct T- and B-cell subsets in RAG1–/– hosts that mirrored young and aged immune systems. Importantly, aged HSCs treated with CASIN reestablished an immune system similar to that of young animals, and thus capable of mounting a strong immune response to vaccination. Our studies further imply that epigenetic signatures already imprinted in aged HSCs determine the transc...
Source: Blood - August 9, 2018 Category: Hematology Authors: Leins, H., Mulaw, M., Eiwen, K., Sakk, V., Liang, Y., Denkinger, M., Geiger, H., Schirmbeck, R. Tags: Hematopoiesis and Stem Cells, Immunobiology and Immunotherapy Source Type: research

Hematopoiesis and the bacterial microbiome
Recent studies have revealed that the intestinal bacterial microbiome plays an important role in the regulation of hematopoiesis. A correlation between adverse hematologic effects and imbalance of the intestinal microbiome, or dysbiosis, is evident in several human conditions, such as inflammatory bowel disease, obesity, and, critically, in the setting of antibiotic exposure. Here we review the effects of gut dysbiosis on the hematological compartment and our current understanding of the mechanisms through which changes in the bacterial microbiome affect hematopoiesis. (Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Yan, H., Baldridge, M. T., King, K. Y. Tags: Hematopoiesis and Stem Cells, Blood Spotlight Source Type: research

Endosome trafficking: blood and more
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Kupfer, G. M. Tags: Free Research Articles COMMENTS Source Type: research

TPO-logy accepted
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Hoffmeister, K. M. Tags: Free Research Articles COMMENTS Source Type: research

Detailing the genomic landscape of myeloma
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Bergsagel, P. L., Kuehl, W. M. Tags: Free Research Articles COMMENTS Source Type: research

CASIN the joint: immune aging at the stem cell level
(Source: Blood)
Source: Blood - August 9, 2018 Category: Hematology Authors: Pawelec, G. P. Tags: Free Research Articles COMMENTS Source Type: research

Morphology and flow cytometry of atypical basophils
(Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Tormey, C. A., Siddon, A. J. Tags: Free Research Articles, BloodWork, Myeloid Neoplasia, Phagocytes, Granulocytes, and Myelopoiesis BLOOD WORK Source Type: research

Atypical basophilia
(Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Boiten, H.-J., de Jongh, E. Tags: Free Research Articles, BloodWork, Myeloid Neoplasia, Phagocytes, Granulocytes, and Myelopoiesis BLOOD WORK Source Type: research

Short course of bortezomib in anemic patients with relapsed cold agglutinin disease: a phase 2 prospective GIMEMA study
(Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Rossi, G., Gramegna, D., Paoloni, F., Fattizzo, B., Binda, F., DAdda, M., Farina, M., Lucchini, E., Mauro, F. R., Salvi, F., Marchetti, M., Fazi, P., Zaja, F., Barcellini, W. Tags: Red Cells, Iron, and Erythropoiesis, Clinical Trials and Observations Letter to Blood Source Type: research

Genetic subtyping of breast implant-associated anaplastic large cell lymphoma
(Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Oishi, N., Brody, G. S., Ketterling, R. P., Viswanatha, D. S., He, R., Dasari, S., Mai, M., Benson, H. K., Sattler, C. A., Boddicker, R. L., McPhail, E. D., Bennani, N. N., Harless, C. A., Singh, K., Clemens, M. W., Medeiros, L. J., Miranda, R. N., Feldma Tags: Lymphoid Neoplasia Letter to Blood Source Type: research

Shear-induced integrin signaling in platelet phosphatidylserine exposure, microvesicle release, and coagulation
It is currently unclear why agonist-stimulated platelets require shear force to efficiently externalize the procoagulant phospholipid phosphatidylserine (PS) and release PS-exposed microvesicles (MVs). We reveal that integrin outside-in signaling is an important mechanism for this requirement. PS exposure and MV release were inhibited in β3–/– platelets or by integrin antagonists. The impaired MV release and PS exposure in β3–/– platelets were rescued by expression of wild-type β3 but not a Gα13 binding–deficient β3 mutant (E733EE to AAA), which blocks outside-in sign...
Source: Blood - August 2, 2018 Category: Hematology Authors: Pang, A., Cui, Y., Chen, Y., Cheng, N., Delaney, M. K., Gu, M., Stojanovic-Terpo, A., Zhu, C., Du, X. Tags: Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research

A CD19/CD3 bispecific antibody for effective immunotherapy of chronic lymphocytic leukemia in the ibrutinib era
The Bruton tyrosine kinase inhibitor ibrutinib induces high rates of clinical response in chronic lymphocytic leukemia (CLL). However, there remains a need for adjunct treatments to deepen response and to overcome drug resistance. Blinatumomab, a CD19/CD3 bispecific antibody (bsAb) designed in the BiTE (bispecific T-cell engager) format, is approved by the US Food and Drug Administration for the treatment of relapsed or refractory B-cell precursor acute lymphoblastic leukemia. Because of its short half-life of 2.1 hours, blinatumomab requires continuous intravenous dosing for efficacy. We developed a novel CD19/CD3 bsAb in...
Source: Blood - August 2, 2018 Category: Hematology Authors: Robinson, H. R., Qi, J., Cook, E. M., Nichols, C., Dadashian, E. L., Underbayev, C., Herman, S. E. M., Saba, N. S., Keyvanfar, K., Sun, C., Ahn, I. E., Baskar, S., Rader, C., Wiestner, A. Tags: Immunobiology and Immunotherapy, Lymphoid Neoplasia Source Type: research

HSP110 sustains chronic NF-{kappa}B signaling in activated B-cell diffuse large B-cell lymphoma through MyD88 stabilization
In conclusion, we identified HSP110 as a regulator of NF-B signaling through MyD88 stabilization in ABC-DLBCL. This finding reveals HSP110 as a new potential therapeutic target in ABC-DLBCL. (Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Boudesco, C., Verhoeyen, E., Martin, L., Chassagne-Clement, C., Salmi, L., Mhaidly, R., Pangault, C., Fest, T., Ramla, S., Jardin, F., Wolz, O.-O., Weber, A. N. R., Garrido, C., Jego, G. Tags: Lymphoid Neoplasia Source Type: research

Dexpramipexole as an oral steroid-sparing agent in hypereosinophilic syndromes
Hypereosinophilic syndromes (HESs) are a heterogeneous group of disorders characterized by peripheral eosinophilia and eosinophil-related end organ damage. Whereas most patients respond to glucocorticoid (GC) therapy, high doses are often necessary, and side effects are common. Dexpramipexole (KNS-760704), an orally bioavailable synthetic aminobenzothiazole, showed an excellent safety profile and was coincidentally noted to significantly decrease absolute eosinophil counts (AECs) in a phase 3 trial for amyotrophic lateral sclerosis. This proof-of-concept study was designed to evaluate dexpramipexole (150 mg orally twice da...
Source: Blood - August 2, 2018 Category: Hematology Authors: Panch, S. R., Bozik, M. E., Brown, T., Makiya, M., Prussin, C., Archibald, D. G., Hebrank, G. T., Sullivan, M., Sun, X., Wetzler, L., Ware, J., Fay, M. P., Dunbar, C. E., Dworetzky, S. I., Khoury, P., Maric, I., Klion, A. D. Tags: Phagocytes, Granulocytes, and Myelopoiesis, Clinical Trials and Observations Source Type: research

How I treat myelofibrosis after failure of JAK inhibitors
The introduction of JAK inhibitors, leading to regulatory approval of ruxolitinib, represents a major therapeutic advance in myelofibrosis (MF). Most patients experience reduction in splenomegaly and improved quality of life from symptom improvement. It is a paradox, however, that, despite inhibition of signaling downstream of disease-related driver mutations, JAK inhibitor treatment is not associated with consistent molecular or pathologic responses in MF. Furthermore, there are important limitations to JAK inhibitor therapy including development of dose-limiting cytopenias and/or nonhematological toxicities such as neuro...
Source: Blood - August 2, 2018 Category: Hematology Authors: Pardanani, A., Tefferi, A. Tags: How I Treat, Free Research Articles, Myeloid Neoplasia, Clinical Trials and Observations Source Type: research

Extracellular galectins as controllers of cytokines in hematological cancer
Galectins and cytokines are both secreted proteins whose levels are prognosis factors for several cancers. Extracellular galectins bind to the glycans decorating glycoproteins and are overproduced in most cancers. Accumulative evidence shows that galectins regulate cytokines during cancer progression. Although galectins alter cytokine function by binding to the glycans decorating cytokines or their receptors, cytokines could also regulate galectin expression and function. This review revises these complex interactions and their clinical impact, particularly in hematological cancers. (Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Gordon-Alonso, M., Bruger, A. M., van der Bruggen, P. Tags: Immunobiology and Immunotherapy, Myeloid Neoplasia, Lymphoid Neoplasia, Blood Spotlight Source Type: research

Identification of a new VHL exon and complex splicing alterations in familial erythrocytosis or von Hippel-Lindau disease
This study demonstrates that cryptic exon retention and exon skipping are new VHL alterations and reveals a novel complex splicing regulation of the VHL gene. These findings open new avenues for diagnosis and research regarding the VHL-related hypoxia-signaling pathway. (Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Lenglet, M., Robriquet, F., Schwarz, K., Camps, C., Couturier, A., Hoogewijs, D., Buffet, A., Knight, S. J. L., Gad, S., Couve, S., Chesnel, F., Pacault, M., Lindenbaum, P., Job, S., Dumont, S., Besnard, T., Cornec, M., Dreau, H., Pentony, M., Kvikstad, E Tags: Pediatric Hematology, Plenary Papers, Red Cells, Iron, and Erythropoiesis Source Type: research

Lets shear a moment, then take it inside
(Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: Ware, J. Tags: Free Research Articles COMMENTS Source Type: research

Platelet lipidomics and function: joining the dots
(Source: Blood)
Source: Blood - August 2, 2018 Category: Hematology Authors: McFadyen, J. D., Peter, K. Tags: Free Research Articles COMMENTS Source Type: research