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Volume 2, Issue 21

 

Cover Image

Cover Figure: Principal component analysis of the top 500 high-variance genes revealed a clustering of Waldenström macroglobulinemia (WM) samples, regardless of MYD88 and CXCR4 mutation status. The finding may explain the overlapping disease characteristics observed among WM patients.
See the article by Hunter et al.

WASHINGTON, November 13,  2018 – 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.

 

In this issue, we present a Point-Counterpoint about when to initiate treatment for patients with smoldering myeloma. Mateos and González-Calle argue for early treatment, whereas Kumar suggests delaying treatment until progression. We hope you find this discussion interesting for your practice. Please share your opinion on the matter using Remarq, an online collaboration tool for readers to make public comments.

 

Castleman disease is a rare lymphoproliferative disorder with variable prognosis. The disease can present as unicentric and patients are generally mildly symptomatic. Multicentric disease is often associated with human herpesvirus 8 infection and carries a more adverse prognosis. In an Exceptional Case Report, Byun and colleagues describe a family with both unicentric and multicentric Castleman disease associated with a novel FAS mutation. The report sheds new light on this disorder.

 

Testing for minimal residual disease has been developed as an important tool in prognosis and determination of treatment durations. Molecular testing for lymphoid diseases has been a sensitive, powerful, and reproducible assay. A central question is what compartment samples are the most representative of disease burden and therefore should be acquired. Mazzotti and colleagues assessed the concordance of studies from the bone marrow and peripheral blood from patients with multiple myeloma and reached interesting findings.

 

Zebrafish have proven to be an outstanding model for the study of hematopoiesis because these animals develop rapidly and blood formation can be directly visualized. In the Review Article. Wattrus and Zon leaders in this area, discuss the hematopoietic niche in zebrafish. They offer novel insights and potential therapeutic targets.

 

The question “Who should get long-term anticoagulant therapy for venous thromboembolism and with what?” is frequently posed in the clinic. Rodger and Le Gal comprehensively address this challenge in a review.

 

In Waldenström macroglobulinemia, activating MYD88 mutations are found in ~95% of cases. Much less is known about those patients with Waldenström macroglobulinemia that is wild type at this locus. Hunter and colleagues in-depth description of 18 patients with this disorder adds to our understanding and provides clues into novel drug approaches.

 

Systemic mastocytosis is a rare hematological disorder with variable prognosis. Pardanani and colleagues studied a cohort of 580 patients with this disorder, using sophisticated molecular and clinical parameters. This is an extraordinary comprehensive analysis of patients with systemic mastocytosis that allowed the development of risk models for future reference.

 

It is well recognized that thrombosis is more common during a number of inflammatory conditions, such as sepsis and autoimmune conditions. The mechanisms underlying this clinical observation have not been clearly elucidated. Salamah and colleagues explore the role of a novel ligand LL37, which may provide an important link and a therapeutic target.

 

Venous thromboembolism (VTE) frequently occurs in cancer patients and is associated with an inferior outcome. Gade and colleagues evaluated the Danish National Chronic Lymphocytic Leukemia (CLL) Registry for VTE. This study precisely defines the incidence of VTE in patients with CLL and highlights important clinical parameters, enhancing prospects for superior patient outcomes.

 

There is ongoing debate as to the relative merits of reduced-intensity and myelobative conditioning for the treatment of patients with hematological malignancies undergoing allogeneic transplantation.  Chhabra and colleagues addressed this question with regard to the treatment of patients with chronic myelogenous leukemia and made interesting findings.

Featured Visual Abstract

High-resolution mapping of the polyclonal immune response to the human platelet alloantigen HPA-1a (PlA1)

Antibodies to platelet antigens result in serious bleeding disorders. One such antigen is HPA-1a/1b (also known as PIA1/A2) is responsible for ~80% of the cases of fetal and neonatal alloimmune thrombocytopenia. Newman and colleagues dissect high-resolution mapping of the immune response to this alloantigen, achieving a better understanding this important clinical immune response. This understanding enhances prospects to develop potential reagents for diagnosis and therapy.

 

 

 

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.