| Summary: | Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is a mature B-cell neoplasm that usually follows an indolent course. In a subset of cases (2-7%), transformation to a higher grade lymphoma occurs (Richter syndrome (RS)). Herein, we report an unusual case of Richter transformation to a composite classical Hodgkin lymphoma (CHL) and anaplastic plasmacytoma in the same left axillary lymph node from a patient who was diagnosed with CLL/SLL seven years prior. The diagnosis of CHL was based on the presence of Reed-Sternberg (R-S) cells and mononuclear variants that showed CD30 and CD15 staining in a membranous and Golgi pattern, weak PAX-5 staining, and negativity for CD20 and CD45. The anaplastic plasmacytoma component comprised of CD138+, CD20 − malignant cells with lambda light chain restriction. Due to the absence of available tissue, clonality studies could not be performed. However, a single clonal origin was highly suggested, as the CLL/SLL component (CD5+, CD19+, CD20+, CD23+, and partial FMC-7+) also demonstrated lambda light chain restriction by flow cytometric analysis. In this article, we describe the clinical and pathological characteristics of this unique case and review the pertinent literature.
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