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 gamma 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.
Liu
Xiao-Yuan
XY
Department of Pathology, Children's Hospital and Research Center at Oakland, Oakland, California 94609-1809, USA. sliu@mail.cho.org
Atkins
Robert C
RC
Feusner
James H
JH
Rowland
Jon M
JM
D000293:Adolescent; D015704:CD4 Antigens / Q000032:analysis; D019002:CD56 Antigen / Q000032:analysis; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics*; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics* / Q000276:immunology / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology
D015704:CD4 Antigens; D019002:CD56 Antigen