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Extramedullary Waldenström macroglobulinemia.
Ranjit Banwait1, Yosra Aljawai, Joseph Cappuccio
1Medical Oncology, Dana-Farber Cancer Institute, and Harvard Medical School, Boston, Massachusetts.
American Journal of Hematology
|October 29, 2014
Summary
Extramedullary disease (EMD) occurs in 4.4% of Waldenstrom Macroglobulinemia (WM) patients, often developing after therapy. This study details EMD characteristics, revealing pulmonary and central nervous system involvement, and provides survival data for this rare WM manifestation.
Area of Science:
- Hematology
- Oncology
- Clinical Medicine
Background:
- Waldenstrom Macroglobulinemia (WM) diagnosis relies on bone marrow B-cell neoplasm percentage and serum IgM paraprotein levels.
- Extramedullary disease (EMD) in WM is infrequently assessed, and its diagnostic and prognostic significance remains unclear.
Purpose of the Study:
- To characterize the clinical features, presentation, and outcomes of patients with extramedullary Waldenstrom Macroglobulinemia (WM).
Main Methods:
- Retrospective analysis of 985 WM patients treated at a single academic center.
- Assessment of EMD presence, sites of involvement, and correlation with patient survival.
Main Results:
- EMD was identified in 4.4% of WM patients (43 individuals).
- EMD manifested at diagnosis in 21% and post-therapy in 79% of cases.
- Common EMD sites included pulmonary (30%), cerebrospinal fluid (23%), and soft tissue (21%).
- The median 10-year overall survival for patients with EMD was 79%.
Conclusions:
- Extramedullary disease is an uncommon but significant feature in Waldenstrom Macroglobulinemia.
- Understanding EMD characteristics is crucial for comprehensive WM patient management.
- Further research into the molecular underpinnings of EMD in WM is warranted.
