Cordua S, Kjaer L, Skov V, Pallisgaard N, Hasselbalch HC, Ellervik C. Prevalence and phenotypes of JAK2 V617F and calreticulin mutations in a Danish general population. Blood. 2019;134(5):469-479.
(Source: Blood)
Source: Blood - October 2, 2019 Category: Hematology Tags: Free Research Articles ERRATUM Source Type: research

Lymphocytes in Sanfilippo syndrome display characteristic Alder-Reilly anomaly
(Source: Blood)
Source: Blood - October 2, 2019 Category: Hematology Authors: Do, L., Pasalic, L. Tags: Free Research Articles, BloodWork BLOOD WORK Source Type: research

A method for noninvasive prenatal diagnosis of monogenic autosomal recessive disorders
(Source: Blood)
Source: Blood - October 2, 2019 Category: Hematology Authors: Cutts, A., Vavoulis, D. V., Petrou, M., Smith, F., Clark, B., Henderson, S., Schuh, A. Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis Letter to Blood Source Type: research

PHF19 promotes multiple myeloma tumorigenicity through PRC2 activation and broad H3K27me3 domain formation
Dysregulation of polycomb repressive complex 2 (PRC2) promotes oncogenesis partly through its enzymatic function for inducing trimethylation of histone H3 lysine 27 (H3K27me3). However, it remains to be determined how PRC2 activity is regulated in normal and diseased settings. We here report a PRC2-associated cofactor, PHD finger protein 19 (PHF19; also known as polycomb-like 3), as a crucial mediator of tumorigenicity in multiple myeloma (MM). Overexpression and/or genomic amplification of PHF19 is found associated with malignant progression of MM and plasma cell leukemia, correlating to worse treatment outcomes. Using va...
Source: Blood - October 2, 2019 Category: Hematology Authors: Ren, Z., Ahn, J. H., Liu, H., Tsai, Y.-H., Bhanu, N. V., Koss, B., Allison, D. F., Ma, A., Storey, A. J., Wang, P., Mackintosh, S. G., Edmondson, R. D., Groen, R. W. J., Martens, A. C., Garcia, B. A., Tackett, A. J., Jin, J., Cai, L., Zheng, D., Wang, G. Tags: Multiple Myeloma, Lymphoid Neoplasia Source Type: research

LMO2 activation by deacetylation is indispensable for hematopoiesis and T-ALL leukemogenesis
Hematopoietic transcription factor LIM domain only 2 (LMO2), a member of the TAL1 transcriptional complex, plays an essential role during early hematopoiesis and is frequently activated in T-cell acute lymphoblastic leukemia (T-ALL) patients. Here, we demonstrate that LMO2 is activated by deacetylation on lysine 74 and 78 via the nicotinamide phosphoribosyltransferase (NAMPT)/sirtuin 2 (SIRT2) pathway. LMO2 deacetylation enables LMO2 to interact with LIM domain binding 1 and activate the TAL1 complex. NAMPT/SIRT2-mediated activation of LMO2 by deacetylation appears to be important for hematopoietic differentiation of induc...
Source: Blood - October 2, 2019 Category: Hematology Authors: Morishima, T., Krahl, A.-C., Nasri, M., Xu, Y., Aghaallaei, N., Findik, B., Klimiankou, M., Ritter, M., Hartmann, M. D., Gloeckner, C. J., Stefanczyk, S., Lindner, C., Oswald, B., Bernhard, R., Hähnel, K., Hermanutz-Klein, U., Ebinger, M., Handgre Tags: Lymphoid Neoplasia Source Type: research

The KDM4/JMJD2 histone demethylases are required for hematopoietic stem cell maintenance
KDM4/JMJD2 are H3K9- and H3K36-specific demethylases, which are considered promising therapeutic targets for the treatment of acute myeloid leukemia (AML) harboring MLL translocations. Here, we investigate the long-term effects of depleting KDM4 activity on normal hematopoiesis to probe potential side effects of continuous inhibition of these enzymes. Utilizing conditional Kdm4a/Kdm4b/Kdm4c triple-knockout mice, we show that KDM4 activity is required for hematopoietic stem cell (HSC) maintenance in vivo. The knockout of the KDM4 demethylases leads to accumulation of H3K9me3 on transcription start sites and the correspondin...
Source: Blood - October 2, 2019 Category: Hematology Authors: Agger, K., Nishimura, K., Miyagi, S., Messling, J.-E., Rasmussen, K. D., Helin, K. Tags: Hematopoiesis and Stem Cells, Myeloid Neoplasia, Brief Reports Source Type: research

Pembrolizumab in relapsed or refractory Hodgkin lymphoma: 2-year follow-up of KEYNOTE-087
We present the 2-year follow-up of the phase 2 KEYNOTE-087 study of pembrolizumab in 210 patients, based on HL progression after autologous stem cell transplantation (ASCT) and subsequent brentuximab vedotin (BV; cohort 1); salvage chemotherapy and BV, with ineligibility for SCT owing to chemorefractory disease (cohort 2); and progression after SCT without BV (cohort 3). With a median follow-up of 27.6 months, the objective response rate (ORR) by blinded independent central review was 71.9% (95% CI, 65.3-77.9), the complete response rate (CRR) was 27.6%, and the partial response (PR) rate was 44.3%. Median duration of resp...
Source: Blood - October 2, 2019 Category: Hematology Authors: Chen, R., Zinzani, P. L., Lee, H. J., Armand, P., Johnson, N. A., Brice, P., Radford, J., Ribrag, V., Molin, D., Vassilakopoulos, T. P., Tomita, A., von Tresckow, B., Shipp, M. A., Lin, J., Kim, E., Nahar, A., Balakumaran, A., Moskowitz, C. H. Tags: Lymphoid Neoplasia, Clinical Trials and Observations Source Type: research

Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia
T-cell prolymphocytic leukemia (T-PLL) is a rare, mature T-cell neoplasm with a heterogeneous clinical course. With the advent of novel treatment options that will potentially change the management of patients with T-PLL, it has become necessary to produce consensus guidelines for the design and conduct of clinical trials. The T-PLL International Study group (TPLL-ISG) set out to define standardized criteria for diagnosis, treatment indication, and evaluation of response. These criteria will facilitate comparison of results from clinical trials in T-PLL, and will thus support clinical decision making, as well as the approv...
Source: Blood - October 2, 2019 Category: Hematology Authors: Staber, P. B., Herling, M., Bellido, M., Jacobsen, E. D., Davids, M. S., Kadia, T. M., Shustov, A., Tournilhac, O., Bachy, E., Zaja, F., Porkka, K., Hoermann, G., Simonitsch-Klupp, I., Haferlach, C., Kubicek, S., Mayerhoefer, M. E., Hopfinger, G., Jaeger, Tags: Special Reports, Free Research Articles, Lymphoid Neoplasia Source Type: research

Tissue factor pathway inhibitor primes monocytes for antiphospholipid antibody-induced thrombosis
Antiphospholipid antibodies (aPLs) with complex lipid and/or protein reactivities cause complement-dependent thrombosis and pregnancy complications. Although cross-reactivities with coagulation regulatory proteins contribute to the risk for developing thrombosis in patients with antiphospholipid syndrome, the majority of pathogenic aPLs retain reactivity with membrane lipid components and rapidly induce reactive oxygen species-dependent proinflammatory signaling and tissue factor (TF) procoagulant activation. Here, we show that lipid-reactive aPLs activate a common species-conserved TF signaling pathway. aPLs dissociate an...
Source: Blood - October 2, 2019 Category: Hematology Authors: Müller-Calleja, N., Hollerbach, A., Ritter, S., Pedrosa, D. G., Strand, D., Graf, C., Reinhardt, C., Strand, S., Poncelet, P., Griffin, J. H., Lackner, K. J., Ruf, W. Tags: Plenary Papers, Thrombosis and Hemostasis Source Type: research

Myelomas sound of silencing
(Source: Blood)
Source: Blood - October 2, 2019 Category: Hematology Authors: Boise, L. H. Tags: Free Research Articles COMMENTS Source Type: research

Pembrolizumab: living up to expectations
(Source: Blood)
Source: Blood - October 2, 2019 Category: Hematology Authors: Ansell, S. M. Tags: Free Research Articles COMMENTS Source Type: research

T-PLL: harmonizing criteria for research
(Source: Blood)
Source: Blood - October 2, 2019 Category: Hematology Authors: Risitano, A. M. Tags: Free Research Articles COMMENTS Source Type: research

Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019;133(16):1703-1714.
(Source: Blood)
Source: Blood - September 25, 2019 Category: Hematology Tags: Free Research Articles ERRATA Source Type: research

Stock W, Luger SM, Advani AS, et al. A pediatric regimen for older adolescents and young adults with acute lymphoblastic leukemia: results of CALGB 10403. Blood. 2019;133(14):1548-1559.
(Source: Blood)
Source: Blood - September 25, 2019 Category: Hematology Tags: Free Research Articles ERRATA Source Type: research

B-lymphoblastic leukemia/lymphoma with an unusual appendiceal involvement
(Source: Blood)
Source: Blood - September 25, 2019 Category: Hematology Authors: Sukswai, N., Thirabanjasak, D. Tags: Free Research Articles, BloodWork, Lymphoid Neoplasia BLOOD WORK Source Type: research