What is the morbidity and mortality of Waldenström macroglobulinemia?

Updated: May 29, 2020
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

The most important causes of death in Waldenström macroglobulinemia include progression of the proliferative process, infection, cardiac failure, renal failure, strokes, and GI bleeding. Transformation to a more aggressive immunoblastic variant is less common (6% of cases).

Treon et al reported that somatic mutations in MYD88 and CXCR4 in patients with Waldenström macroglobulinemia are determinants of clinical presentation and overall survival. Patients with MYD88 (L265P) and CXCR4 with warts, hypogammaglobulinemia, infections and myelokathexis (WHIM) syndrome/nonsense (NS) mutations had significantly higher bone marrow disease involvement and symptomatic disease requiring therapy, including hyperviscosity syndrome. [21]

Patients with MYD88 (L265P) and WHIM/frameshift (FS) CXCR4 mutations or wild type CXCR4 had intermediate bone marrow involvement, while those with wild type MYD88 and CXCR4 had the lowest bone marrow disease burden. Risk of death was not affected by CXCR4 mutation status, but was markedly increased by wild type MYD88 status (hazard ratio 10.54). [21]


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