Histologic Transformation in an Untreated Waldenstrom's Macroglobulinemia After 14 Years: Case Report and Review of the Literature.

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.