title: CD4+/CD56+ hematodermic neoplasm: report of a rare variant with a T-cell receptor gene rearrangement.
abstract: CD4+/CD56+ hematodermic neoplasm (HN), formerly known as a blastic natural killer (NK) cell lymphoma, is a rare subtype of a cutaneous dendritic cell neoplasm notable for highly aggressive behavior. The characteristic features are: expression of the T-helper/inducer cell marker CD4 and the NK-cell marker CD56 in the absence of other T cell or NK-cell specific markers. In particular, CD3 (surface or cytoplasmic) and CD2 are not expressed. Although T-cell receptor (TCR) genes are generally reported to be in a germline configuration, we present an unusual variant of a CD4+/CD56+ HN with a clonal rearrangement of TCR genes. This feature of a CD4+/CD56+ HN has been only rarely reported. Recognition of the presence of clonal TCR gene rearrangements in a small subset of CD4+/CD56+ HN is important to avoid misdiagnosis of this entity as an unusual variant of a cutaneous T-cell lymphoma.
authors.affiliation: Department of Pathology, University of Washington, Seattle, WA 98195-6100, USA. gstetsen@u.washington.edu
authors.forename: Galina Y
authors.initials: GY
authors.lastname: Stetsenko
authors.forename: Rob
authors.initials: R
authors.lastname: McFarlane
authors.forename: Andrea
authors.initials: A
authors.lastname: Kalus
authors.forename: John
authors.initials: J
authors.lastname: Olerud
authors.forename: Sindhu
authors.initials: S
authors.lastname: Cherian
authors.forename: Jonathan
authors.initials: J
authors.lastname: Fromm
authors.forename: Evan
authors.initials: E
authors.lastname: George
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Argenyi
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004273:DNA, Neoplasm / Q000235:genetics; D003907:Dexamethasone / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D005434:Flow Cytometry; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics*; D006801:Humans; D007150:Immunohistochemistry; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014408:Biomarkers, Tumor; D015704:CD4 Antigens; D019002:CD56 Antigen; D004273:DNA, Neoplasm; D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D003520:Cyclophosphamide