CD20-Positive and CD4/CD8-Double-Negative Peripheral T-Cell Lymphoma of Spleen Complicated with Severe Disseminated Intravascular Coagulation and Enteropathy

A 71-year-old woman presented with massive splenomegaly. Open splenectomy was performed, and the diagnosis of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), was made, with a characteristic immunophenotype of CD3+, CD4-, CD8-, T-cell receptor (TCR)αβ+, and TCRγδ-. After splenectomy, she suffered abrupt exacerbation of the lymphoma with disseminated intravascular coagulation and enteropathy. Although chemotherapy was started, her medical condition did not improve and she died a week later. Postmortem reevaluation of the pathological specimen confirmed her diagnosis as CD20+ PTCL-NOS. Although it is a rare disease entity, CD20+ T-cell lymphoma can demonstrate aggressive clinical behavior. [J Clin Exp Hematopathol 52(2) : 133-136, 2012]