Blastic NK‐cell‐like lymphoma with T‐cell receptor gene rearrangement

Blastic natural killer (NK) cell lymphoma is very rare but has been recently classified as a distinct entity in WHO classification. However, the classification remains controversial, and the clinicopathologic spectrum is not completely understood. We report a unique case of cutaneous CD4 + CD56 + malignancy with a typical clinical presentation and immunophenotype of blastic NK‐cell lymphoma in a 15‐year‐old Guamanian girl. The skin was the only site involved by the lymphoma. Molecular study showed clonal T‐cell receptor γ gene rearrangement. The patient has been disease‐free till now (more than 12 months following bone marrow transplant). This case may represent a tumor at an early stage of a common developmental pathway for T‐cells and NK‐cells. Am. J. Hematol. 75:251–253, 2004. © 2004 Wiley‐Liss, Inc.