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BLOOD COMMENTARIES

SPECIAL REPORT

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.

MYELOID NEOPLASIA

Short telomere syndromes are premature aging syndromes associated with a wide range of phenotypes. The authors report on their experience with cancer (predominantly myelodysplastic syndrome and acute myeloid leukemia) in persons with these syndromes.

RED CELLS, IRON, AND ERYTHROPOIESIS

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.

THROMBOSIS AND HEMOSTASIS

About 2.5% of protein S is synthesized in megakaryocytes and packaged in platelet granules, but its function is not clear. Calzavarini and colleagues demonstrated that platelet protein S is important in limiting venous thrombosis.

TRANSFUSION MEDICINE

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.

LETTERS TO BLOOD

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.

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.

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.

Breast implant–associated anaplastic large-cell lymphoma is a rare T-cell lymphoma that arises adjacent to breast implants. The authors describe 3 patients with Epstein-Barr virus–positive (EBV+) diffuse large B-cell lymphoma as a rare subtype of breast implant–related lymphoma.

BLOOD WORK

ERRATUM

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