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Indian Journal of Medical and Paediatric Oncology
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CASE REPORT
Year : 2020  |  Volume : 41  |  Issue : 4  |  Page : 596-601

Waldenström macroglobulinemia: A clinicopathological profile and review of six cases


1 Department of Clinical Hematology, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Clinical Hematology, Nilratan Sircar Medical College, Kolkata, West Bengal, India

Correspondence Address:
Dr. Priyanka Samal
Department of Clinical Hematology, Institute of Medical Sciences and SUM Hospital, K8 Kalinga Nagar, Shampur, Bhubaneswar - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmpo.ijmpo_234_19

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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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