How I treat polycythemia vera
Since its discovery, polycythemia vera (PV) has challenged clinicians responsible for its diagnosis and management and scientists investigating its pathogenesis. As a clonal hematopoietic stem cell (HSC) disorder, PV is a neoplasm but its driver mutations result in overproduction of morphologically and functionally normal blood cells. PV arises in an HSC but it can present initially as isolated erythrocytosis, leukocytosis, thrombocytosis, or any combination of these together with splenomegaly or myelofibrosis, and it can take years for a true panmyelopathy to appear. PV shares the same JAK2 mutation as essential thrombocy...
Source: Blood - July 24, 2019 Category: Hematology Authors: Spivak, J. L. Tags: Hematopoiesis and Stem Cells, How I Treat, Myeloid Neoplasia Source Type: research
NETing the mechanism of inflammation in DADA2
(Source: Blood)
Source: Blood - July 24, 2019 Category: Hematology Authors: Lee, P. Y. Tags: Free Research Articles COMMENTS Source Type: research
Help from outside: cysteine to survive in AML
(Source: Blood)
Source: Blood - July 24, 2019 Category: Hematology Authors: Stuani, L., Sarry, J.-E. Tags: Free Research Articles COMMENTS Source Type: research
Make time for gait speed: vital to staging the aging
(Source: Blood)
Source: Blood - July 24, 2019 Category: Hematology Authors: Wildes, T. M. Tags: Free Research Articles COMMENTS Source Type: research
Targeting GSK3: beyond the mitotic spindle?
(Source: Blood)
Source: Blood - July 24, 2019 Category: Hematology Authors: Steidl, C. Tags: Free Research Articles COMMENTS Source Type: research
Pediatric chronic graft-versus-host disease
(Source: Blood)
Source: Blood - July 17, 2019 Category: Hematology Tags: Free Research Articles, CME article CONTINUING MEDICAL EDUCATION (CME) QUESTIONS Source Type: research
Brady JL, Binkley MS, Hajj C, et al. Definitive radiotherapy for localized follicular lymphoma staged by 18F-FDG PET-CT: a collaborative study by ILROG. Blood. 2019;133(3):237-245.
(Source: Blood)
Source: Blood - July 17, 2019 Category: Hematology Tags: Free Research Articles ERRATUM Source Type: research
"Double-hit" follicular lymphoma
(Source: Blood)
Source: Blood - July 17, 2019 Category: Hematology Authors: Xu, S., Huang, Q. Tags: Free Research Articles, BloodWork, Lymphoid Neoplasia BLOOD WORK Source Type: research
Cuplike nuclear morphology is highly associated with IKZF1 deletion in pediatric precursor B-cell ALL
(Source: Blood)
Source: Blood - July 17, 2019 Category: Hematology Authors: Li, W., Cooley, L. D., August, K. J., Richardson, A. I., Shao, L., Ahmed, A. A., Farooqi, M. S., Zwick, D. L. Tags: Pediatric Hematology, Lymphoid Neoplasia Letter to Blood Source Type: research
Simple, reliable detection of amyloid in fat aspirates using the fluorescent dye FSB: prospective study in 206 patients
(Source: Blood)
Source: Blood - July 17, 2019 Category: Hematology Authors: Tasaki, M., Milani, P., Foli, A., Verga, L., Obici, L., Basset, M., Bozzola, M., Ferraro, G., Nuvolone, M., Morbini, P., Capello, G., Ueda, M., Obayashi, K., Paulli, M., Ando, Y., Merlini, G., Palladini, G., Lavatelli, F. Tags: Multiple Myeloma, Lymphoid Neoplasia, Clinical Trials and Observations Letter to Blood Source Type: research
Timing of inhibitor development in more than 1000 previously untreated patients with severe hemophilia A
(Source: Blood)
Source: Blood - July 17, 2019 Category: Hematology Authors: van den Berg, H. M., Fischer, K., Carcao, M., Chambost, H., Kenet, G., Kurnik, K., Königs, C., Male, C., Santagostino, E., Ljung, R., on behalf of the PedNet Study Group Tags: Pediatric Hematology, Thrombosis and Hemostasis, Clinical Trials and Observations Letter to Blood Source Type: research
Benefits and challenges with diagnosing chronic and late acute GVHD in children using the NIH consensus criteria
The objectives of this prospective multi-institution study were to determine: (1) whether the NIH-CC could be used to diagnose pediatric cGVHD and whether the criteria operationalize well in a multi-institution study; (2) the frequency of cGVHD and L-aGVHD in children using the NIH-CC; and (3) the clinical features and risk factors for cGVHD and L-aGVHD using the NIH-CC. Twenty-seven transplant centers enrolled 302 patients <18 years of age before conditioning and prospectively followed them for 1 year posttransplant for development of cGVHD. Centers justified their cGVHD diagnosis according to the NIH-CC using central ...
Source: Blood - July 17, 2019 Category: Hematology Authors: Cuvelier, G. D. E., Nemecek, E. R., Wahlstrom, J. T., Kitko, C. L., Lewis, V. A., Schechter, T., Jacobsohn, D. A., Harris, A. C., Pulsipher, M. A., Bittencourt, H., Choi, S. W., Caywood, E. H., Kasow, K. A., Bhatia, M., Oshrine, B. R., Flower, A., Chaudhu Tags: Pediatric Hematology, Transplantation, Free Research Articles, CME article Source Type: research
Plasmin-mediated fibrinolysis enables macrophage migration in a murine model of inflammation
Efficient migration of macrophages to sites of inflammation requires cell surface–bound plasmin(ogen). Here, we investigated the mechanisms underlying the deficits of plasmin(ogen)-mediated macrophage migration in 2 models: murine thioglycollate-induced peritonitis and in vitro macrophage migration. As previously reported, macrophage migration into the peritoneal cavity of mice in response to thioglycollate was significantly impaired in the absence of plasminogen. Fibrin(ogen) deposition was noted in the peritoneal cavity in response to thioglycollate, with a significant increase in fibrin(ogen) in the plasminogen-de...
Source: Blood - July 17, 2019 Category: Hematology Authors: Silva, L. M., Lum, A. G., Tran, C., Shaw, M. W., Gao, Z., Flick, M. J., Moutsopoulos, N. M., Bugge, T. H., Mullins, E. S. Tags: Phagocytes, Granulocytes, and Myelopoiesis, Thrombosis and Hemostasis Source Type: research
EFL1 mutations impair eIF6 release to cause Shwachman-Diamond syndrome
Shwachman-Diamond syndrome (SDS) is a recessive disorder typified by bone marrow failure and predisposition to hematological malignancies. SDS is predominantly caused by deficiency of the allosteric regulator Shwachman-Bodian-Diamond syndrome that cooperates with elongation factor-like GTPase 1 (EFL1) to catalyze release of the ribosome antiassociation factor eIF6 and activate translation. Here, we report biallelic mutations in EFL1 in 3 unrelated individuals with clinical features of SDS. Cellular defects in these individuals include impaired ribosomal subunit joining and attenuated global protein translation as a consequ...
Source: Blood - July 17, 2019 Category: Hematology Authors: Tan, S., Kermasson, L., Hoslin, A., Jaako, P., Faille, A., Acevedo-Arozena, A., Lengline, E., Ranta, D., Poiree, M., Fenneteau, O., Ducou le Pointe, H., Fumagalli, S., Beaupain, B., Nitschke, P., Bole-Feysot, C., de Villartay, J.-P., Bellanne-Chantelot, C Tags: Hematopoiesis and Stem Cells, Myeloid Neoplasia, Red Cells, Iron, and Erythropoiesis Source Type: research
Hepatic leukemia factor is a novel leukemic stem cell regulator in DNMT3A, NPM1, and FLT3-ITD triple-mutated AML
FLT3, DNMT3A, and NPM1 are the most frequently mutated genes in cytogenetically normal acute myeloid leukemia (AML), but little is known about how these mutations synergize upon cooccurrence. Here we show that triple-mutated AML is characterized by high leukemia stem cell (LSC) frequency, an aberrant leukemia-specific GPR56highCD34low immunophenotype, and synergistic upregulation of Hepatic Leukemia Factor (HLF). Cell sorting based on the LSC marker GPR56 allowed isolation of triple-mutated from DNMT3A/NPM1 double-mutated subclones. Moreover, in DNMT3A R882-mutated patients, CpG hypomethylation at the HLF transcription sta...
Source: Blood - July 17, 2019 Category: Hematology Authors: Garg, S., Reyes-Palomares, A., He, L., Bergeron, A., Lavallee, V.-P., Lemieux, S., Gendron, P., Rohde, C., Xia, J., Jagdhane, P., Müller-Tidow, C., Lipka, D. B., Imren, S., Humphries, R. K., Waskow, C., Vick, B., Jeremias, I., Richard-Carpentier, Tags: Myeloid Neoplasia Source Type: research
