Blastic CD4 NK cell leukemia/lymphoma: a distinct clinical entity.
We report the findings of three new cases of a distinct clinicopathologic natural killer (NK) cell malignancy characterized by cutaneous, nodal and bone marrow infiltration by CD3-CD4+CD56+ NK blastic cells. Tumor cells were detected in bone marrow and in peripheral blood smears and showed finely distributed nuclear chromatin with nucleoli and a moderate amount of cytoplasm. Epstein-Barr virus (EBV) DNA was negative in the two tested cases. The immunophenotypes determined by flow cytometry were identical concerning mCD3-cytCD3-CD4+weakCD56+ HLA-DR+. The TCR was in germline configuration in the two cases tested. NK cell activity was demonstrated only in one out of the two cases tested. The negative reactions with alpha-naphthyl-acetate-esterase (ANAE), CD11b and CD14 strongly suggested that the tumor cells were not of the monocytic lineage.
Department of Clinical Medicine, School of Medicine Ribeirão Preto-USP, Ribeirão Preto, SP, Brazil. rpfalcao@fmrp.usp.br
Roberto P
RP
Falcão
Aglair B
AB
Garcia
Maria G
MG
Marques
Belinda P
BP
Simões
Benedito A
BA
Fonseca
Miriam L
ML
Rodrigues
Norma T
NT
Foss
D000293:Adolescent; D000368:Aged; D000951:Antigens, Neoplasm / Q000032:analysis; D019002:CD56 Antigen / Q000032:analysis; D002648:Child; D006684:HLA-DR Antigens / Q000032:analysis; D004854:Herpesvirus 4, Human; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology*; D007945:Leukemia, Lymphoid / Q000145:classification* / Q000276:immunology / Q000473:pathology; D017254:Leukemic Infiltration; D008206:Lymphatic Diseases / Q000473:pathology; D008223:Lymphoma / Q000145:classification* / Q000276:immunology / Q000473:pathology; D008297:Male; D014411:Neoplastic Stem Cells / Q000473:pathology*; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000235:genetics; D012867:Skin / Q000473:pathology; D013163:Splenomegaly / Q000473:pathology
D000951:Antigens, Neoplasm; D019002:CD56 Antigen; D006684:HLA-DR Antigens; D016692:Receptors, Antigen, T-Cell, gamma-delta