Increased SLAMF7high monocytes in myelofibrosis patients harboring JAK2V617F provide a therapeutic target of elotuzumab
In conclusion, an increase of SLAMF7high monocytes with higher JAK2V617F allele burden was associated with the onset of MF in MPN patients harboring JAK2V617F, and Elo could be a therapeutic agent for MPN patients with MF who harbor JAK2V617F. (Source: Blood)
Source: Blood - September 4, 2019 Category: Hematology Authors: Maekawa, T., Kato, S., Kawamura, T., Takada, K., Sone, T., Ogata, H., Saito, K., Izumi, T., Nagao, S., Takano, K., Okada, Y., Tachi, N., Teramoto, M., Horiuchi, T., Hikota-Saga, R., Endo-Umeda, K., Uno, S., Osawa, Y., Kobayashi, A., Kobayashi, S., Sato, K Tags: Myeloid Neoplasia Source Type: research

Integrative genomic analysis identifies key pathogenic mechanisms in primary mediastinal large B-cell lymphoma
Primary mediastinal large B-cell lymphoma (PMBL) represents a clinically and pathologically distinct subtype of large B-cell lymphomas. Furthermore, molecular studies, including global gene expression profiling, have provided evidence that PMBL is more closely related to classical Hodgkin lymphoma (cHL). Although targeted sequencing studies have revealed a number of mutations involved in PMBL pathogenesis, a comprehensive description of disease-associated genetic alterations and perturbed pathways is still lacking. Here, we performed whole-exome sequencing of 95 PMBL tumors to inform on oncogenic driver genes and recurrent...
Source: Blood - September 4, 2019 Category: Hematology Authors: Mottok, A., Hung, S. S., Chavez, E. A., Woolcock, B., Telenius, A., Chong, L. C., Meissner, B., Nakamura, H., Rushton, C., Vigano, E., Sarkozy, C., Gascoyne, R. D., Connors, J. M., Ben-Neriah, S., Mungall, A., Marra, M. A., Siebert, R., Scott, D. W., Sava Tags: Lymphoid Neoplasia Source Type: research

Early progression as a predictor of survival in marginal zone lymphomas: an analysis from the FIL-NF10 study
Marginal zone lymphomas (MZLs) are indolent nonfollicular B-cell lymphomas (INFLs) and have heterogeneous clinical behavior. Recently, time to progression of disease at 24 months (POD24) was identified to stratify overall survival (OS) in follicular non-Hodgkin lymphoma and in INFL. Here, we examined the ability of POD24 to predict subsequent OS in a large, international cohort of MZL as part of the NF10 prospective international registry headed by Fondazione Italiana Linfomi (FIL). POD24 was only calculated for MZL patients requiring immediate therapy and was defined as experiencing lymphoma progression within 24 months f...
Source: Blood - September 4, 2019 Category: Hematology Authors: Luminari, S., Merli, M., Rattotti, S., Tarantino, V., Marcheselli, L., Cavallo, F., Varettoni, M., Bianchi, B., Merli, F., Tedeschi, A., Cabras, G., Re, F., Visco, C., Torresan Delamain, M., Cencini, E., Spina, M., Ferrero, S., Ferrari, A., Deodato, M., M Tags: Lymphoid Neoplasia, Brief Reports, Clinical Trials and Observations Source Type: research

Genetic defects in hematopoietic transcription factors and predisposition to acute lymphoblastic leukemia
Recent genome-wide studies have revealed a plethora of germline variants that significantly influence the susceptibility to acute lymphoblastic leukemia (ALL), thus providing compelling evidence for genetic inheritance of this blood cancer. In particular, hematopoietic transcription factors (eg, ETV6, PAX5, IKZF1) are most frequently implicated in familial ALL, and germline variants in these genes confer strong predisposition (albeit with incomplete penetrance). Studies of germline risk factors for ALL provide unique insights into the molecular etiology of this leukemia. (Source: Blood)
Source: Blood - September 4, 2019 Category: Hematology Authors: Gocho, Y., Yang, J. J. Tags: Lymphoid Neoplasia, Blood Spotlight Source Type: research

Teamwork makes the dream work in thrombopoiesis
(Source: Blood)
Source: Blood - September 4, 2019 Category: Hematology Authors: Asquith, N. L., Machlus, K. R. Tags: Free Research Articles COMMENTS Source Type: research

SLAM dunk for myelofibrosis?
(Source: Blood)
Source: Blood - September 4, 2019 Category: Hematology Authors: Marcellino, B. K., Mascarenhas, J. Tags: Free Research Articles COMMENTS Source Type: research

PMBL: flying under the immune radar
(Source: Blood)
Source: Blood - September 4, 2019 Category: Hematology Authors: Rimsza, L. M. Tags: Free Research Articles COMMENTS Source Type: research

POD24 in MZL: a means to an end or an end point in itself?
(Source: Blood)
Source: Blood - September 4, 2019 Category: Hematology Authors: Maurer, M. J. Tags: Free Research Articles COMMENTS Source Type: research

A gelatinous leukemia
(Source: Blood)
Source: Blood - August 28, 2019 Category: Hematology Authors: Lefebvre, T., Mauvieux, L. Tags: Hematopoiesis and Stem Cells, Free Research Articles, BloodWork, Lymphoid Neoplasia BLOOD WORK Source Type: research

Primitive macrophages are dispensable for HSPC mobilization and definitive hematopoiesis
(Source: Blood)
Source: Blood - August 28, 2019 Category: Hematology Authors: Yuan, H., Gao, S., Chen, H., Liu, X., Zhou, J., de The, H., Zhu, J. Tags: Hematopoiesis and Stem Cells Letter to Blood Source Type: research

Activated protein C ameliorates chronic graft-versus-host disease by PAR1-dependent biased cell signaling on T cells
Soluble thrombomodulin plasma concentrations are elevated in steroid-resistant graft-versus-host disease (GVHD), implying endothelial hypofunctioning for thrombomodulin-dependent generation of activated protein C’s (APC) anticoagulant, anti-inflammatory, and antiapoptotic functions. Recombinant thrombomodulin or APC administration decreases acute GVHD, manifested by intense inflammation and tissue destruction. Here, we administered recombinant murine wild-type (WT) APC to mice with established chronic GVHD (cGVHD), a less-inflammatory autoimmune-like disease. WT APC normalized bronchiolitis obliterans–induced p...
Source: Blood - August 28, 2019 Category: Hematology Authors: Sinha, R. K., Flynn, R., Zaiken, M., Paz, K., Gavin, A. L., Nemazee, D., Fernandez, J. A., Xu, X., Griffin, J. H., Blazar, B. R. Tags: Transplantation, Brief Reports Source Type: research

Platelet hyaluronidase-2 regulates the early stages of inflammatory disease in colitis
In this study, we provide evidence that HA catabolism is disrupted in human intestinal microvascular endothelial cells isolated from patients with IBD. Furthermore, mice deficient in HYAL2 are more susceptible to an acute model of colitis, and this increased susceptibility is abrogated by transfusion of HYAL2-competent platelets. Finally, we show that platelets, via HYAL2-dependent degradation of endothelial HA, regulate the early stages of inflammation in colitis by limiting leukocyte extravasation. (Source: Blood)
Source: Blood - August 28, 2019 Category: Hematology Authors: Petrey, A. C., Obery, D. R., Kessler, S. P., Zawerton, A., Flamion, B., de la Motte, C. A. Tags: Immunobiology and Immunotherapy, Platelets and Thrombopoiesis Source Type: research

Early progression after bendamustine-rituximab is associated with high risk of transformation in advanced stage follicular lymphoma
Despite widespread use of bendamustine and rituximab (BR) as frontline therapy for advanced-stage follicular lymphoma (FL), little is known about the risk of early progression or incidence of histological transformation. We performed a retrospective analysis of a population-based cohort of 296 patients with advanced-stage FL treated with frontline BR and maintenance rituximab. As previously demonstrated, outcomes with this regimen are excellent, with 2-year event-free survival estimated at 85% (95% confidence interval [95% CI], 80-89) and 2-year overall survival 92% (95% CI, 88-95). Progression of disease within 24 months ...
Source: Blood - August 28, 2019 Category: Hematology Authors: Freeman, C. L., Kridel, R., Moccia, A. A., Savage, K. J., Villa, D. R., Scott, D. W., Gerrie, A. S., Ferguson, D., Cafferty, F., Slack, G. W., Farinha, P., Skinnider, B., Connors, J. M., Sehn, L. H. Tags: Lymphoid Neoplasia, Brief Reports, Clinical Trials and Observations Source Type: research

Fertility preservation in patients with hematologic malignancies and recipients of hematopoietic cell transplants
Patients with hematologic malignancies and those undergoing hematopoietic cell transplantation (HCT) face a complex set of challenges when considering options for fertility preservation (FP). There are no standard options for prepubertal children, and women with hematologic malignancies may not be eligible for standard FP options. Fortunately, initial therapies for most blood cancers are not highly gonadotoxic, affording an important opportunity for postremission counseling and referrals to fertility specialists. These patients face a high risk of relapse, and many will be referred for autologous or allogeneic HCT, which c...
Source: Blood - August 28, 2019 Category: Hematology Authors: Loren, A. W., Senapati, S. Tags: Transplantation, Review Articles Source Type: research

Availability of FLT3 inhibitors: how do we use them?
The natural history of FLT3-mutated AML is changing after the approval of midostaurin for frontline therapy and gilteritinib for relapsed or refractory patients. Recently reported, positive randomized trials of the drugs gilteritinib, quizartinib, and sorafenib predict even wider use of FLT3 inhibitors going forward. FLT3 inhibitors now emerge as an important, if not indispensable, part of therapy for a large subset of high-risk patients. (Source: Blood)
Source: Blood - August 28, 2019 Category: Hematology Authors: Perl, A. E. Tags: Myeloid Neoplasia, Blood Spotlight, Clinical Trials and Observations Source Type: research