Extramedullary disease in Waldenström macroglobulinemia: A population-based observational study

Area of Science:

• Hematology
• Oncology

Background:

• Extramedullary disease (EMD) is an uncommon manifestation of Waldenström macroglobulinemia (WM).
• The clinical course and prognostic value of EMD in WM are not well-established.

Purpose of the Study:

• To investigate the clinical significance and prognostic implications of EMD in a Waldenström macroglobulinemia cohort.
• To determine the incidence and affected sites of EMD in WM patients.

Main Methods:

• A single-center study analyzed a cohort of 469 patients with Waldenström macroglobulinemia.
• Clinical data and outcomes were retrospectively reviewed to identify EMD and assess its impact.

Main Results:

• Extramedullary disease (EMD) was diagnosed in 30 (6.4%) Waldenström macroglobulinemia patients.
• The central nervous system, kidneys, and lungs were the most common sites for EMD.
• The cumulative incidence of EMD reached 12.6% at 15 years, with median overall survival rates of 63% and 37% at 5 and 10 years, respectively, for patients with EMD.

Conclusions:

• Extramedullary disease (EMD) presents a persistent risk throughout the course of Waldenström macroglobulinemia.
• EMD was not found to significantly impact long-term survival in this cohort of WM patients.