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Case Reports

Abstract

Primary Cutaneous Marginal Zone Lymphoma (PCMZL) with admixed Hodgkin Lymphoma (HL) cells is an exceptionally rare collision. Even HL concomitant with non-Hodgkin lymphoma (NHL) in the same patient is very unusual and significant as the treatment and prognosis of these entities differ. We report a case of this rare collision in a 71-year-old female with a history of Merkel cell carcinoma who presented with skin lesion on mid-back and lymphadenopathy. An excisional biopsy of the mid-back lesion and subsequently of right inguinal lymph node was performed. Histologic examination of the lymph node displayed a diffuse and focally nodular cellular proliferation surrounded by thick sclerotic bands. The cellular proliferation was composed of scattered large mononucleated, binucleated and multinucleated cells [Reed-Sternberg (RS) and Lacunar cells] with distinct nucleoli and abundant cytoplasm in the background of small lymphocytes, histiocytes, and plasma cells. Immunohistochemistry was positive for CD30, CD15, MUM1, and dim positive for PAX5. Overall findings were diagnostic of Nodular Sclerosis HL. Skin biopsy from mid-back demonstrated diffuse and nodular proliferation of mixed small and large atypical lymphoid cells. Scattered plasma cells and large, atypical cells, morphologically resembling RS cells were noted. Focal areas with abundant apoptosis and coagulative necrosis were present. FISH was negative for BCL2 and c-MYC rearrangement. Kappa and Lambda ISH showed Kappa-restricted monotypic plasma cells, consistent with PCMZL. The scattered large cells and RS cells were positive for CD30, CD15, PAX5, MUM1, and EBER, confirming admixed HL. To our knowledge, this collision has never been reported earlier in literature.