- Special Report: new consensus recommendations for diagnosis and treatment of Erdheim-Chester disease
- F cells are genetically nearly identical to their adult A erythroblasts
- Viral infection or inflammatory disease may block efficacy of RhIg in preventing alloimmunization
- Adjunct hydroxyurea for stroke prevention in children with SCD during the COVID-19 pandemic
- Pattern of clonal hematopoiesis following therapy of mantle cell lymphoma
- Blood Podcast, Episode 22
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In this week’s episode we will review a report on the use of hydroxyurea as an alternative to transfusion therapy for the prevention of recurrent strokes in patients with sickle cell anemia, learn more about why some erythroblasts continue to produce hemoglobin F in adults, and review a study that explores cancer outcomes in patients with short telomere syndromes.
Erdheim-Chester disease: consensus recommendations for evaluation, diagnosis, and treatment in themolecular era
andLong-term follow-up of mTOR inhibition for Erdheim-Chester disease天天三张牌安卓版
Two articles this week focus on Erdheim-Chester disease (ECD), a rare histiocytosis that mainly affects adults. Clonal somatic mutations primarily involving proteins in the BRAF and MPAK pathways have established ECD as a myeloid neoplasm, with targeted therapies now available for patients. In the first paper, an international panel presents new consensus recommendations for evaluation and treatment of ECD. In the second paper, Pegoraro and colleagues present long-term outcomes of patients with ECD treated with sirolimus, with responses in patients both with and without BRAF mutations.
Understanding heterogeneity of fetal hemoglobin induction through comparative analysis of F and A erythroblasts天天三张牌安卓版
Increasing fetal hemoglobin expression is an important clinical approach to the treatment of hemoglobinopathies, but the mechanism of upregulation of hemoglobin F (HbF) is still not clear. Khandros et al demonstrated by elegant transcriptional and proteomic profiling studies that F cells and A cells are virtually identical, suggesting that HbF expression and nonexpression reflect isolated transcriptional changes in the β-globin locus..
Poly(I:C) causes failure of immunoprophylaxis to red blood cells expressing the KEL glycoprotein in mice天天三张牌安卓版
Anti-D therapy prevents hemolytic disease of the newborn in about 98% of Rh-negative women, but the remainder may have breakthrough alloimmunization. The authors demonstrated that inflammation can decrease the efficacy of immunoprophylaxis, suggesting that inflammatory disease or viral infection may lead to failure of Rh immunoglobulin in preventing alloimmunization.
Initiating adjunct low-dose hydroxyurea therapy for stroke prevention in children with SCA during the COVID-19 pandemic天天三张牌安卓版In anticipation of possible blood shortages during the current COVID-19 pandemic, DeBaun proposes rapid initiation of administration of low, fixed doses of hydroxyurea for children with sickle cell anemia (SCA) who receive regular prophylactic transfusions for stroke prevention.
Clonal hematopoiesis evolves from pretreatment clones and stabilizes after end of chemotherapy in patients with MCL天天三张牌安卓版
Eskelund et al examined clonal hematopoiesis (CH) in a cohort of patients with mantle cell lymphoma (MCL) treated with first-line chemotherapy and autologous stem cell transplantation. In young, good-risk MCL patients, CH after first-line therapy arises almost entirely from preexisting clones, stabilizes after a period of expansion posttransplantation, and does not negatively impact survival..