Cases of cutaneous and nasal CD56 (NCAM)-positive lymphoma in Japan have differences in immunohistology, genotype, and etiology

CD56 (NCAM)-positive lymphoma frequently involves the skin and nasal area. This study shows it is likely that the clinicopathologic features of this l ymphoma are distinctive to each of die primarily involved sites. Sixteen cu taneous and 11 nasal cases of CD56-positive lymphoma were examined. In 10 c utaneous cases, the lesions consisted of pleomorphic large-cell lymphoma th at expressed CD3 epsilon (CD3), CD2 (LFA2), CD4, CD122 (IL-2 beta receptor) , TIA1, perforin, and granzyme B and displayed angiocentric/angiodestructiv e features. Lobular panniculitis was found in 8 of these cases, and 6 cases showed other organ involvement. The remaining 6 cutaneous cases consisted mostly of CD3 epsilon- and CD4-positive, CD2-, CD122-, and TIA1-negative la rge blastic lymphoma, having less angiodestruction and panniculitis. Bone m arrow invasion and leukemic changes were found in 4 of these cases during t he clinical course. All 11 nasal cases showed pleomorphic small and medium- size lymphoma cells with angiodestructive features and were positive for CD 3 epsilon, CD2, CD122, TIA1, perforin, and granzyme B. CD4-positive lymphom a ic as found in 4 of these cases. Only 3 nasal cases showed other organ in volvement. Genotypically, 2 of the 4 cases examined in the first cutaneous group, 3 of the 4 cases examined in the second cutaneous group, and only 1 of the 11 nasal cases showed rearrangement of the TcRC beta gene by the Sou thern blot method. Only 2 cutaneous cases with panniculitis and all 11 nasa l cases showed a positive nuclear signal for EBV-encoded RNA (EBERs) by in situ hybridization. Thus, two types of cutaneous CD56-positive lymphoma wer e found, each having a unique cell characteristic, genotype, and EBV infect ion pattern that differed from that of nasal-type lymphoma. HUM PATHOL 30:1 024-1034. Copyright (C) 1999 by W.B. Saunders Company.