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Diffuse Large B-Cell Lymphoma Occurring in Patients With Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia
Clinicopathologic Features of 12 Cases

Pei Lin MD, Adnan Mansoor MD, Carlos Bueso-Ramos MD, PhD, Suyang Hao MD, Raymond Lai MD, PhD, L. Jeffrey Medeiros MD
DOI: http://dx.doi.org/10.1309/R01VXG46MFCDVNHL 246-253 First published online: 1 August 2003

Abstract

Of 92 patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) treated at our institution, diffuse large B-cell lymphoma (DLBCL) also developed in 12 (13%). In 10 patients, DLBCL developed 12 to 128 months (median, 44 months) after the diagnosis of LPL/WM. Two patients had LPL/WM and DLBCL simultaneously. Clinicopathologic features at diagnosis of LPL/WM did not predict the risk of DLBCL. Onset of DLBCL was characterized by worsening constitutional symptoms, profound cytopenias, extramedullary disease, and organomegaly. Immunoglobulin light chain expression was identical in both LPL/WM and DLBCL. In situ hybridization for Epstein-Barr virus (EBV) in 8 cases of DLBCL was negative. Of 11 patients with clinical follow-up information available, 8 (73%) died within 10 months of diagnosis of DLBCL. DLBCL, most likely as a result of histologic transformation, occurs in a subset of patients with LPL/WM and is associated with aggressive clinical course and poor outcome. EBV is unlikely to be involved in transformation.

Key Words:
  • Large cell lymphoma
  • Lymphoplasmacytic lymphoma
  • Waldenström macroglobulinemia