A cutaneous agranular CD2- CD4+ CD56+ 'lymphoma': Report of two cases and review of the literature

Junichi Kameoka, Ryo Ichinohasama, Misako Tanaka, Ikuo Miura, Yosuo Tomiya, Shinichiro Takahashi, Minami Yamada, Izumi Ishikawa, Ikuko Kadowaki, Osamu Sasaki, Jim Kimura, Kuniaki Meguro, Kiyoshi Ooya, Sadayoshi Ito

Research output: Contribution to journalArticlepeer-review

59 Citations (Scopus)


We report 2 cases of agranular CD2- CD4+ CD56+ non-Hodgkin lymphoma in which skin seemed to be the primary site. A 21-year-old woman's initial symptom was a skin nodule on the right cheek. She also had tumors in the nasopharynx, and the bone marrow subsequently became involved. No lymphadenopathy was present. She experienced complete remission after dose- intensified therapy with cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone (CHOP), but the disease relapsed in the central nervous system 6 months later. An 81-year-old man experienced an 11-month history of skin nodules in the left forearm. On admission, he had a bone marrow infiltration of lymphoma cells. He died of pneumonia during chemotherapy. The malignant cells of the 2 patients had similar morphologic features, with a monocytoid nucleus and no cytoplasmic granules. The cells in both cases showed a unique phenotype: CD2-, CD3-, CD4+, CD8-, CD13-, CD14-, CD34-, CD16-, CD56+, CD57-, HLA-DR-positive. Staining for peroxidase and α-naphthyl butyrate esterase was negative. The T-cell receptor β, γ, δ, IgH, κ, λ genes were of germ line configurations. The DNA of Epstein-Barr virus was not detected from the bone marrow cells by polymerase chain reaction. Only 3 other cases with similar phenotypes have been reported; all had skin lesions. Although the origin of these cells remains unknown, we propose that this is a distinct clinicopathologic entity.

Original languageEnglish
Pages (from-to)478-488
Number of pages11
JournalAmerican Journal of Clinical Pathology
Issue number4
Publication statusPublished - 1998 Oct


  • CD4
  • CD56
  • Malignant lymphoma
  • Skin

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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