Waldenstrom's macroglobulinemia in the era of immunotherapy.

Abstract:

:Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma that presents with symptomatic anemia, thrombocytopenia, constitutional symptoms, extramedullary disease and rarely hyperviscosity syndrome. The presence of both IgM monoclonal protein and ≥10% monoclonal lymphoplasmacytic cells is required for the diagnosis. MyD88 is present in 67-90% of patients but is not pathognomonic for WM. Many patients who fulfill the criteria of WM are asymptomatic and do not require treatment. Recent advances in the understanding of the biology of WM have paved the way for new treatment options. The use of novel agents with or without rituximab enables the use of effective chemotherapy-free regiments upfront and in the relapsed setting. New targeted treatments such as venetoclax and CXCR4 antagonists are being investigated.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Vaxman I,Gertz M

doi

10.1080/10428194.2020.1711901

subject

Has Abstract

pub_date

2020-06-01 00:00:00

pages

1292-1304

issue

6

eissn

1042-8194

issn

1029-2403

journal_volume

61

pub_type

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