Composite Lymphoma as Co-occurrence of Advanced Chronic Lymphocytic Leukemia / Small Lymphocytic Lymphoma Carrying Trisomy 12 and t(14;18) and Peripheral T-cell Lymphoma

• Published in: Journal of Clinical and Experimental Hematopathology Vol. 58; no. 1; pp. 27 - 31
• Main Authors: Yumi Aoyama, Taiichi Kodaka, Yuriko Zushi, Yuta Goto, Hiroko Tsunemine, Tomoo Itoh, Takayuki Takahashi
• Format: Journal Article
• Language: Japanese
• Published: The Japanese Society for Lymphoreticular Tissue Research 01.03.2018
• ISSN: 1346-4280

Composite lymphoma is defined as the co-occurrence of two types of lymphoma, comprising 1 - 4% of lymphomas, and the association of B-cell-type chronic lymphocytic leukemia (B-CLL)/small lymphocytic lymphoma and peripheral T-cell lymphoma (PTCL) is rare. Here, we report a case (77-year-old woman) of advanced B-CLL complicated by newly appearing PTCL. Two years after the onset of B-CLL, CLL cells acquired CD38 antigen expression and the disease entity became CLL/prolymphocytic leukemia. Trisomy 12 and t(14;18) karyotypes were observed. Five years after the onset of B-CLL, large abnormal cells with convoluted nuclei appeared in the peripheral blood and rapidly increased in number. These cells were positive for CD3, CD4, CD5, CD30 (partially), CD56, and αβ-type T-cell receptor (TCR), in which PCR demonstrated monoclonal TCR-γ gene rearrangement. An additional diagnosis of PTCL, not otherwise specified was made. We treated her with an R-CHOP regimen, resulting in the marked reduction of B-CLL cells but progressive PTCL. Brentuximab vedotin had a transient effect, but the patient died of sepsis due to residual PTCL and pancytopenia. This case is highly informative for tumor biology of B-CLL in terms of emergence of both chromosomal abnormalities and PTCL with progression of this leukemia.