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Volume 1, Issue 16

 

Cover Image

Cover Figure: The JH1 domain of the JAK2 protein. The catalytic loop is highlighted in cyan, the αC loop in yellow, and phosphotyrosines within the activation loop in green. See the article by Lee et al.

WASHINGTON, July 11,  2017 – Welcome to the “Advance Notice,”  newsletter which provides highlights from issues of Blood Advances, the open-access journal of the American Society of Hematology (ASH), that  are hand-picked by Blood Advances Editor-in-Chief Robert Negrin, MD.

 

Maintenance therapy with lenalidomide is a standard approach to prolong remissions following therapy for patients with multiple myeloma. Rarely, secondary malignancies, especially myelodysplastic syndrome (MDS) and acute myeloid leukemia, have been observed. Miller et al present an interesting case of a patient who developed MDS that reversed after cessation of this drug.

Thrombotic microangiopathy is a rare but potentially devastating complication of hematopoietic stem cell transplantation. Treatment is difficult and often supportive after removing offending agents such as calcineurin inhibitors. Goodship and colleagues present a case of a 4-year-old boy who developed this complication and was successfully treated with the complement inhibitor offering additional treatment considerations for this challenging case.

Osteonecrosis of the femoral head is a relatively common complication in patients with sickle cell disease. Adesina et al evaluate the incidence of this problem in patients in California between 1991 and 2013. This analysis sets the stage for future interventions to try to prevent or treat this devastating complication.

It is well recognized that genetics can impact venous thromboembolism (VTE); however, the majority of patients do not have a known heritable mutation. In the paper by Lee et al, 64 patients with VTE were evaluated with whole-exome sequencing and a 55-gene thrombophilia panel was developed. This panel was found to identify a potential disease-causing genetic mutation in the majority of patients analyzed.

Allogeneic hematopoietic cell transplantation for patients with sickle cell disease is a potentially curative treatment approach. However, many patients do not have an HLA-matched sibling donor. Gilman et al developed an approach using mismatched family or unrelated donors with excellent outcomes, albeit in a limited number of patients. These results could form the basis of a larger study to broaden the donor pool for this patient population.

Featured Visual Abstract

WNT ligands contribute to the immune response during septic shock and amplify endotoxemia-driven inflammation in mice

Shiva Keshava, Jagan Sundaram, Anuradha Rajulapati, Charles T. Esmon, Usha R. Pendurthi and L. Vijaya Mohan Rao

 

 

 

Blood Advances is the open-access journal of the American Society of Hematology (ASH) (www.hematology.org), the world’s largest professional society concerned with the causes and treatment of blood disorders.

ASH’s mission is to further the understanding, diagnosis, treatment, and prevention of disorders affecting blood, bone marrow, and the immunologic, hemostatic, and vascular systems by promoting research, clinical care, education, training, and advocacy in hematology.