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

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 kappa light chain restriction by in situ hybridization and also revealed clonal immunoglobulin light chain rearrangements in both kappa and lambda. 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