A cutaneous agranular CD2- CD4+ CD56+ "lymphoma": report of two cases and review of the literature.
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 alpha-naphthyl butyrate esterase was negative. The T-cell receptor beta, gamma, delta, IgH, kappa, lambda 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.
Second Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.
J
J
Kameoka
R
R
Ichinohasama
M
M
Tanaka
I
I
Miura
Y
Y
Tomiya
S
S
Takahashi
M
M
Yamada
I
I
Ishikawa
I
I
Kadowaki
O
O
Sasaki
J
J
Kimura
K
K
Meguro
K
K
Ooya
S
S
Ito
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018801:CD2 Antigens / Q000032:analysis*; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D008228:Lymphoma, Non-Hodgkin / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009303:Nasopharyngeal Neoplasms / Q000188:drug therapy / Q000556:secondary; D011241:Prednisone / Q000627:therapeutic use; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D013120:Spinal Cord Neoplasms / Q000188:drug therapy / Q000556:secondary; D014750:Vincristine / Q000627:therapeutic use
D018801:CD2 Antigens; D015704:CD4 Antigens; D019002:CD56 Antigen; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; C027260:pirarubicin; D011241:Prednisone