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.