Issue Archive

Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.

Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.

Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.

Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.

Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.

Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.

Inhibitor development is a major challenge to treatment of severe hemophilia. Malec and colleagues report on the results of the first prospective study of immune tolerance induction (ITI) using a recombinant factor VIII Fc fusion protein with an extended half-life. The authors found that durable ITI is achieved by a median of 3 months in 10 of 16 patients, setting the stage for further evaluation of this product.

To prevent stem cell exhaustion, hematopoietic stem cells (HSCs) are quiescent and divide rarely; nevertheless, their characteristics change as we age. Hammond et al quantify the proliferation kinetics and cell-cycle progression of single highly purified HSCs isolated from cord blood, young adults, and older healthy donors. The authors show that older HSCs are less sensitive to growth factor stimulation and are more difficult to activate than young HSCs, providing mechanistic insights to explain these changes.

An immune-suppressive tumor microenvironment is a major barrier to the efficacy of antibody-based therapies. Dacek et al present a novel strategy to overcome this by enhancing the antibody-dependent killing of tumors through additional infusion of chimeric antigen receptor (CAR) T cells engineered to locally secrete a small anti-CD47 blocking agent that disrupts antiphagocytic signaling induced by tumor cell CD47 binding to macrophage signal-regulatory protein α. This increases macrophage-mediated antibody-dependent cellular phagocytosis or antibody-dependent cellular cytotoxicity and enables increased tumor cell lysis in a mouse model. Such strategies can now be tested in clinical trials.

In this month’s CME article, Fattizzo and colleagues describe the outcomes of the rare problem of autoimmune hemolytic anemia (AIHA) during pregnancy in a multicenter cohort of 33 patients covering 45 pregnancies. The authors report good maternal outcomes despite severe hemolysis and substantial relapse rates but also reveal a higher rate of serious fetal and neonatal complications than seen in controls. These data inform practice when clinicians are faced with AIHA in a pregnant patient.

Endogenous plasma protein anticoagulants limit clot formation by inhibiting factor IXa activity. Ivanciu and colleagues show that factor-delaying IXa inhibition using novel zymogen-like variants increases the potency of administered factor IX in models of hemophilia B. The authors’ data enhance our understanding of the control of clot formation, and the findings suggest a novel strategy to augment clot formation in hemophilia B.