Abstract
Composite lymphoma (CL) is a rare form of lymphoma composed of two or more distinct types of malignant lymphomas involving a single anatomic location. CL of T and B cells is exceedingly rare. Hairy cell leukemia as part of a composite B- and T-cell lymphoma is rare. We present an unusual case of a CL of peripheral T-cell and B-cell lymphoma with immunophenotype of hairy cell leukemia. A 76-year-old woman presented with left cervical lymphadenopathy. PET scan was negative for other lymphadenopathy or organomegaly. She had a history of treated peripheral T-cell lymphoma in a different node 8 years back. She had neutropenia after chemotherapy. Bone marrow examination was normal. Excisional biopsy of the lymph node revealed architectural effacement by small lymphocytes intermixed with large atypical lymphoid cells demonstrating extensive nuclear irregularities and prominent nucleoli. Flow cytometry showed predominance of monoclonal B cells with expression of CD19, CD20, CD23, CD25, CD11c, and CD103, along with T cells expressing CD2, CD3, CD5, CD7, CD38, and CD4/CD8 ratio of 11:1. Immunohistochemistry revealed a predominant B-cell population with T cells expressing CD15 and CD30 and negative for ALK. PCR assays confirmed clonal rearrangements for T-cell receptor gamma and immunoglobulin heavy chain genes, establishing a diagnosis of composite B- and T-cell lymphoma. This was unusual as the B-cell immunophenotype was consistent with hairy cell leukemia, but the morphology and clinical presentation were not. Furthermore, coexpression of CD15 and CD30 in T-cell lymphoma was interestingly rare. CL of T and B cells poses a diagnostic challenge because one component may overshadow the other and the coexisting T cells are often marked as “reactive.” There are currently no agreed-on standards for treatment. This case highlights the importance of a systemic approach and ancillary techniques to arrive at this challenging diagnosis.
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