Histologic Transformation in an Untreated Waldenstrom’s Macroglobulinemia After 14 Years: Case Report and Review of the Literature
Open Access
- 1 January 2021
- journal article
- review article
- Published by Elmer Press, Inc. in Journal of Hematology
- Vol. 10 (1), 25-29
- https://doi.org/10.14740/jh767
Abstract
Waldenstrom's macroglobulinemia (WM) is an indolent B-cell non-Hodgkin lymphoma characterized by lymphoplasmacytic histology in the bone marrow with monoclonal IgM. Median survival can be in excess of 10 years. The 5-year cumulative incidence of death is low at about 10%. One-third of all-cause specific mortality is due to the lymphoma for which histologic transformation (HT) is rare. Here we present a case of a 60-year-old man with longstanding untreated WM, presenting with minimally symptomatic transformation to diffuse large B-cell lymphoma (DLBCL), with an accompanying review of the literature. Transformed WM, diagnosed greater than 5 years, has a reported survival period of 8 - 9 months. This case highlights that after a decade of continued stability in WM, not requiring treatment, an acute change in laboratory data with minimally progressive IgM levels, in the absence of B symptoms and clinical findings, may be the harbinger of transformation and at the time of diagnosis can have a rapidly deteriorating clinical course. In this case, the tripling of the lactate dehydrogenase (LDH) as the primary drastic change demonstrates the importance of the rapid increase in LDH as a singly reliable marker for HT. Late transformation has been borne out as a negative variable as the generally indolent course of WM is curtailed with the poor outcome in HT. Although MYD88 wildtype is a possible predictive factor for transformation, it is unclear if late transformation is clonally or non-clonally related and further molecular investigation is needed.Keywords
This publication has 29 references indexed in Scilit:
- Cause‐specific mortality in individuals with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia, 2000–2016British Journal of Haematology, 2020
- Diagnosis and Management of Waldenström MacroglobulinemiaJAMA Oncology, 2017
- Waldenström macroglobulinemia: biology, genetics, and therapyBlood and Lymphatic Cancer: Targets and Therapy, 2016
- The 2016 revision of the World Health Organization classification of lymphoid neoplasmsBlood, 2016
- Overall survival and competing risks of death in patients with Waldenström macroglobulinaemia: an analysis of the Surveillance, Epidemiology and End Results databaseBritish Journal of Haematology, 2014
- Heterogeneity of Histological Transformation Events in Waldenström's Macroglobulinemia (WM) and Related DisordersClinical Lymphoma Myeloma and Leukemia, 2011
- Increased Incidence of Transformation and Myelodysplasia/Acute Leukemia in Patients With Waldenström Macroglobulinemia Treated With Nucleoside AnalogsJournal of Clinical Oncology, 2009
- B-cell neoplasms of the lymphocytic, lymphoplasmacytoid, and plasma cell types: Immunohistologic analysis and clinical correlationHuman Pathology, 1985
- Bone Marrow Histology in Waldenström's MacroglobulinaemiaScandinavian Journal of Haematology, 1983
- An Unusual Case of MacroglobulinemiaArchives of Internal Medicine, 1967