title: Clinicopathological analyses of 5 Japanese patients with CD56+ primary cutaneous lymphomas.
abstract: We analyzed the clinicopathological features of 5 Japanese patients with CD56+ primary cutaneous lymphomas (3 men and 2 women aged 25 to 73 years). Except for 1 patient in whom bone marrow involvement was simultaneously observed, all patients presented with cutaneous lesions. Based on their Epstein-Barr virus (EBV) status, we categorized these patients into 2 groups, namely EBV-encoded small RNA-1 (EBER-1) (3 patients) and EBER-1- (2 patients). Generalized lymphadenopathy and bone marrow involvement were observed only in EBER-1 patients. Morphologically, angiocentric proliferation was more prominent in EBER-1+ patients and was accompanied by panniculitis-like changes. The lymphomas in EBER-1- patients featured monomorphic proliferation of lymphoblastic cells with no cytoplasmic granules. Phenotypically, CD3-, cytoplasmic CD3 epsilon+, and CD56+ were common findings in both types. The EBER-1- type showed an additional distinguishing feature, CD7+, CD4+, CD8-, HLA-DR+, and terminal deoxynucleotidyl transferase-positive (TdT+) phenotype. The lymphoma was primarily resistant in the EBER-1+ type, and the patients died within 6 months of admission. In contrast, the lymphoma in the EBER-1- patients was originally chemosensitive. Collectively, we consider there to be at least 2 types of CD56+ primary cutaneous lymphomas, corresponding to nasal-type natural killer (NK)/T-cell lymphomas (EBER-1+) and blastic NK-cell lymphomas (EBER-1-).
authors.affiliation: Division of Hematology, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, 305-8575, Japan. hkojima@md.tsukuba.ac.jp
authors.forename: H
authors.initials: H
authors.lastname: Kojima
authors.forename: H Y
authors.initials: HY
authors.lastname: Mukai
authors.forename: A
authors.initials: A
authors.lastname: Shinagawa
authors.forename: C
authors.initials: C
authors.lastname: Yoshida
authors.forename: M
authors.initials: M
authors.lastname: Kamoshita
authors.forename: T
authors.initials: T
authors.lastname: Komeno
authors.forename: Y
authors.initials: Y
authors.lastname: Hasegawa
authors.forename: Y
authors.initials: Y
authors.lastname: Yamashita
authors.forename: N
authors.initials: N
authors.lastname: Mori
authors.forename: T
authors.initials: T
authors.lastname: Nagasawa
mesh_terms: D000328:Adult; D000368:Aged; D019046:Bone Marrow Neoplasms / Q000209:etiology / Q000473:pathology; D019002:CD56 Antigen; D005260:Female; D006801:Humans; D007564:Japan; D007694:Killer Cells, Natural / Q000473:pathology; D008223:Lymphoma / Q000145:classification / Q000473:pathology* / Q000821:virology*; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000209:etiology / Q000473:pathology / Q000821:virology; D012367:RNA, Viral / Q000097:blood; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology / Q000821:virology*; D013601:T-Lymphocytes / Q000473:pathology
chemical_list: D019002:CD56 Antigen; C099537:Epstein-Barr virus encoded RNA 1; D012367:RNA, Viral