Ebstein-Barr Virus-Negative Clonal Plasma Cell Proliferations Associated With Systemic Involvement of Primary Cutaneous T-Cell Lymphoma

Smita C. Patel, Brett Mahon

Abstract


We describe a 66-year-old man with a long-standing history of squamous cell carcinoma of the head and neck region who developed nodular-sclerosing subtype of classical Hodgkin lymphoma and primary cutaneous CD8-positive, cytotoxic variant of mycosis fungoides over a 1-year period of time. Within a few months of his diagnosis of primary cutaneous T-cell lymphoma, he developed systemic involvement of T-cell lymphoma in an axillary lymph node, bone marrow and lung. Interestingly, his lung infiltrates had an Ebstein-Barr virus (EBV)-negative, mature clonal plasma cell proliferations intermingled with neoplastic T cells. It showed cytoplasmic κ light chain restriction by in situ hybridization and also revealed clonal immunoglobulin light chain rearrangements in both κ and λ. No morphological or immunohistochemical evidence of his prior Hodgkin lymphoma was identified in the bone marrow or lung. In the short available follow-up (2 months to date), the patient is doing well and being evaluated for possible allogeneic stem cell transplant. The association of EBV-positive B-cell expansions in T-cell lymphomas, especially angioimmunoblastic T-cell lymphoma, is well recognized. However, EBV-negative, clonal B-cell or plasma cell proliferations in T-cell lymphoma may represent a specific phenomenon and its significance still needs further clarification.




J Hematol. 2016;5(4):129-137
doi: https://doi.org/10.14740/jh300w


Keywords


Peripheral T-cell lymphomas; Plasma cell proliferations; Clonal; Ebstein-Barr virus

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Journal of Hematology, quarterly, ISSN 1927-1212 (print), 1927-1220 (online), published by Elmer Press Inc.            
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