Waldenström macroglobulinemia: A clinicopathological profile and review of six cases
Waldenström macroglobulinemia (WM) is an indolent B-cell neoplasm in which lymphoplasmacytic cells are accumulated in the bone marrow. This rare disease is incurable with the available treatments and its management is generally based on risk-adapted methods. We present here six cases of WM with thei...
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| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Thieme Medical and Scientific Publishers Pvt. Ltd.
2020-01-01
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| Series: | Indian Journal of Medical and Paediatric Oncology |
| Subjects: | |
| Online Access: | http://www.ijmpo.org/article.asp?issn=0971-5851;year=2020;volume=41;issue=4;spage=596;epage=601;aulast=Samal |
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| Summary: | Waldenström macroglobulinemia (WM) is an indolent B-cell neoplasm in which lymphoplasmacytic cells are accumulated in the bone marrow. This rare disease is incurable with the available treatments and its management is generally based on risk-adapted methods. We present here six cases of WM with their clinicopathological profile and response to therapy. This is a retrospective observational study conducted in a tertiary referral center in Eastern India from October 2014 to December 2016. The clinical manifestations, diagnostic challenges, and response to therapy for WM were analyzed. A total of six cases of WM were analyzed, of which five were male; the mean age was 65 years. The most common presentation was transfusion-dependent anemia, followed by hyperviscosity. All the patients had bone marrow infiltration by lymphoplasmacytoid cells, and serum protein electrophoresis and immunofixation showed immunoglobulin M kappa monoclonal protein. Bortezomib, dexamethasone, and rituximab (BDR) was the most commonly used regimen. Complete and very good partial responses were seen in one patient (16.7%) each and partial response in 3 (50%) patients. There was disease progression to diffuse large B-cell lymphoma in one patient (16.7%). After a 26-month follow-up, four of six patients (66.67%) remained free of disease progression. A very low incidence of WM was reported, and patients had a varied clinicopathological spectrum. A combination of BDR is a promising frontline therapy in the patients of WM. |
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| ISSN: | 0971-5851 0975-2129 |