Dapsone poisoning
(Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: McLeod-Kennedy, L., Leach, M. Tags: Free Research Articles, BloodWork, Red Cells, Iron, and Erythropoiesis BLOOD WORK Source Type: research
Refractory gout in primary myelofibrosis
(Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: Venugopal, S., Mascarenhas, J. Tags: Free Research Articles, BloodWork, Myeloid Neoplasia BLOOD WORK Source Type: research
Two decades of excellent transplant survival for chronic granulomatous disease: a supraregional immunology transplant center report
(Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: Lum, S. H., Flood, T., Hambleton, S., McNaughton, P., Watson, H., Abinun, M., Owens, S., Cigrovski, N., Cant, A., Gennery, A. R., Slatter, M. Tags: Pediatric Hematology, Immunobiology and Immunotherapy, Transplantation, Clinical Trials and Observations Letter to Blood Source Type: research
CRISPR/Cas9 PIG-A gene editing in nonhuman primate model demonstrates no intrinsic clonal expansion of PNH HSPCs
(Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: Shin, T.-H., Baek, E. J., Corat, M. A. F., Chen, S., Metais, J.-Y., AlJanahi, A. A., Zhou, Y., Donahue, R. E., Yu, K.-R., Dunbar, C. E. Tags: Hematopoiesis and Stem Cells, Red Cells, Iron, and Erythropoiesis, Gene Therapy Letter to Blood Source Type: research
Red blood cells modulate structure and dynamics of venous clot formation in sickle cell disease
Sickle cell disease (SCD) is associated with chronic activation of coagulation and an increased risk of venous thromboembolism. Erythrocyte sickling, the primary pathologic event in SCD, results in dramatic morphological changes in red blood cells (RBCs) because of 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. Sickling of RBCs and a significant increase in fibrin deposition were observed in venous thrombi formed in sickle mice. During ex vivo clot contractio...
Source: Blood - June 5, 2019 Category: Hematology Authors: Faes, C., Ilich, A., Sotiaux, A., Sparkenbaugh, E. M., Henderson, M. W., Buczek, L., Beckman, J. D., Ellsworth, P., Noubouossie, D. F., Bhoopat, L., Piegore, M., Renoux, C., Bergmeier, W., Park, Y., Ataga, K. I., Cooley, B., Wolberg, A. S., Key, N. S., Pa Tags: Sickle Cell Disease, Red Cells, Iron, and Erythropoiesis, Thrombosis and Hemostasis Source Type: research
Regulation of gene expression by miR-144/451 during mouse erythropoiesis
The microRNA (miRNA) locus miR-144/451 is abundantly expressed in erythrocyte precursors, facilitating their terminal maturation and protecting against oxidant stress. However, the full repertoire of erythroid miR-144/451 target messenger RNAs (mRNAs) and associated cellular pathways is unknown. In general, the numbers of mRNAs predicted to be targeted by an miRNA vary greatly from hundreds to thousands, and are dependent on experimental approaches. To comprehensively and accurately identify erythroid miR-144/451 target mRNAs, we compared gene knockout and wild-type fetal liver erythroblasts by RNA sequencing, quantitative...
Source: Blood - June 5, 2019 Category: Hematology Authors: Xu, P., Palmer, L. E., Lechauve, C., Zhao, G., Yao, Y., Luan, J., Vourekas, A., Tan, H., Peng, J., Schuetz, J. D., Mourelatos, Z., Wu, G., Weiss, M. J., Paralkar, V. R. Tags: Red Cells, Iron, and Erythropoiesis Source Type: research
Identification of a novel enhancer of CEBPE essential for granulocytic differentiation
CCAAT/enhancer binding protein (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. Analysis of DNase I hypersensitive sites sequencing data revealed an open chromatin region 6 kb downstream of the transcriptional start site of Cebpe in murine myeloid cells. We identified an interaction between this +6-kb region and the core promoter...
Source: Blood - June 5, 2019 Category: Hematology Authors: Shyamsunder, P., Shanmugasundaram, M., Mayakonda, A., Dakle, P., Teoh, W. W., Han, L., Kanojia, D., Lim, M. C., Fullwood, M., An, O., Yang, H., Shi, J., Hossain, M. Z., Madan, V., Koeffler, H. P. Tags: Hematopoiesis and Stem Cells, Phagocytes, Granulocytes, and Myelopoiesis Source Type: research
The chromatin-binding protein Phf6 restricts the self-renewal of hematopoietic stem cells
Recurrent inactivating mutations have been identified in the X-linked plant homeodomain finger protein 6 (PHF6) gene, encoding a chromatin-binding transcriptional regulator protein, in various hematological malignancies. However, the role of PHF6 in normal hematopoiesis and its tumor-suppressor function remain largely unknown. We herein generated mice carrying a floxed Phf6 allele and inactivated Phf6 in hematopoietic cells at various developmental stages. The Phf6 deletion in embryos augmented the capacity of hematopoietic stem cells (HSCs) to proliferate in cultures and reconstitute hematopoiesis in recipient mice. The P...
Source: Blood - June 5, 2019 Category: Hematology Authors: Miyagi, S., Sroczynska, P., Kato, Y., Nakajima-Takagi, Y., Oshima, M., Rizq, O., Takayama, N., Saraya, A., Mizuno, S., Sugiyama, F., Takahashi, S., Matsuzaki, Y., Christensen, J., Helin, K., Iwama, A. Tags: Hematopoiesis and Stem Cells Source Type: research
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 permit progression from the aberrant plasma cell clone to MGUS and overt MM. Additional evidence supports bidirectional interaction of MGUS cells with surrounding cells in the bone marrow niche that regulates malignant transformation. However, there are no robust prognostic biomarkers. Herein we review the current body of literature on the biology of MGUS and provide a rationale for the improve...
Source: Blood - June 5, 2019 Category: Hematology Authors: Mouhieddine, T. H., Weeks, L. D., Ghobrial, I. M. Tags: Multiple Myeloma, Lymphoid Neoplasia, Review Articles Source Type: research
Simplifying the diagnosis of inherited platelet disorders? The new tools do not make it any easier
The molecular causes of many inherited platelet disorders are being unraveled. Next-generation sequencing facilitates diagnosis in 30% to 50% of patients. However, interpretation of genetic variants is challenging and requires careful evaluation in the context of a patient’s phenotype. Before detailed testing is initiated, the treating physician and patient should establish an understanding of why testing is being performed and discuss potential consequences, especially before testing for variants in genes associated with an increased risk for hematologic malignancies. (Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: Greinacher, A., Eekels, J. J. M. Tags: Platelets and Thrombopoiesis, Blood Spotlight Source Type: research
Recommendations for the management of hemophagocytic lymphohistiocytosis in adults
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, whereas 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). Most information on the diagnosis and treatment of HLH comes from the pedia...
Source: Blood - June 5, 2019 Category: Hematology Authors: La Rosee, P., Horne, A., Hines, M., von Bahr Greenwood, T., Machowicz, R., Berliner, N., Birndt, S., Gil-Herrera, J., Girschikofsky, M., Jordan, M. B., Kumar, A., van Laar, J. A. M., Lachmann, G., Nichols, K. E., Ramanan, A. V., Wang, Y., Wang, Z., Janka, Tags: Special Reports, Immunobiology and Immunotherapy, Free Research Articles, Clinical Trials and Observations Source Type: research
Sickle cells and sickle trait in thrombosis
(Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: Kato, G. J. Tags: Free Research Articles COMMENTS Source Type: research
PHF6: it is written in the stem cells
(Source: Blood)
Source: Blood - June 5, 2019 Category: Hematology Authors: Ntziachristos, P. Tags: Free Research Articles COMMENTS Source Type: research
Follicular lymphoma with composite in situ mantle cell neoplasia
(Source: Blood)
Source: Blood - May 29, 2019 Category: Hematology Authors: Subtil, A., Xu, Z. Tags: Free Research Articles, BloodWork, Lymphoid Neoplasia BLOOD WORK Source Type: research
Inhibition of LSD1 by small molecule inhibitors stimulates fetal hemoglobin synthesis
(Source: Blood)
Source: Blood - May 29, 2019 Category: Hematology Authors: Le, C. Q., Myers, G., Habara, A., Jearawiriyapaisarn, N., Murphy, G. J., Chui, D. H. K., Steinberg, M. H., Engel, J. D., Cui, S. Tags: Red Cells, Iron, and Erythropoiesis Letter to Blood Source Type: research
