Waldenström Macroglobulinemia: A Clinicopathological Profile and Review of Six Cases
Open Access
- 1 July 2020
- journal article
- review article
- Published by Georg Thieme Verlag KG in Indian Journal of Medical and Paediatric Oncology
- Vol. 41 (04), 596-601
- https://doi.org/10.4103/ijmpo.ijmpo_234_19
Abstract
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.Keywords
This publication has 21 references indexed in Scilit:
- Serum IgM level as predictor of symptomatic hyperviscosity in patients with Waldenström macroglobulinaemiaBritish Journal of Haematology, 2017
- How I treat Waldenström macroglobulinemiaBlood, 2015
- Waldenstrom macroglobulinemia: prognosis and managementBlood Cancer Journal, 2015
- How to manage Waldenstrom’s macroglobulinemiaLeukemia, 2013
- Response assessment in Waldenström macroglobulinaemia: update from the VIth International WorkshopBritish Journal of Haematology, 2012
- Waldenström macroglobulinemiaBlood, 2007
- Patterns of clinical presentation, treatment, and outcome in patients with Waldenstrom's macroglobulinemia: A two-institution studySeminars in Oncology, 2003
- Clinicopathological definition of Waldenstrom's macroglobulinemia: Consensus Panel Recommendations from the Second International Workshop on Waldenstrom's MacroglobulinemiaSeminars in Oncology, 2003
- WALDENSTRÖM'S MACROGLOBULINEMIAHematology/Oncology Clinics of North America, 1999
- Incidence of Waldenstrom's macroglobulinemiaBlood, 1993