A transitional variant of a T-lymphoproliferative malignancy. Two cases of cutaneous T-cell lymphoma co-expressing E and Fc gamma receptors with final leukaemic transformation.
2 cases of malignant T-lymphoproliferative disease are reported. The proliferating cell was a large blast expressing E and Fc gamma receptors but no helper or suppressor phenotypes and no SmIg. Skin infiltrates were the dominant clinical sign with conspicuous perivascular aggregations of T, E, Fc gamma lymphocytes, though both patients initially had disseminated disease with mild lymphadenopathy, splenomegaly and, in case 2, also hepatic infiltrations. Accordingly, DNA measurements on skin biopsies, taken early in the course, showed a dominating hypotetraploid clone (case 1) and a pronounced population in S-phase (case 2). The patients were alive for 6 and 2 yr, respectively, with a final fatal course of about 6 months duration involving a rather sudden progression of the skin infiltrates, increasing lymphadenopathy and splenomegaly, leukaemic transformation of the neoplastic T, E, Fc gamma lymphocyte and practically no response to cytostatic treatment.
D000368:Aged; D000945:Antigens, Differentiation, T-Lymphocyte; D000954:Antigens, Surface / Q000032:analysis; D001853:Bone Marrow / Q000473:pathology; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000473:pathology*; D008875:Middle Aged; D011961:Receptors, Fc / Q000032:analysis; D017452:Receptors, IgG; D011971:Receptors, Immunologic / Q000032:analysis; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000276:immunology*
D000945:Antigens, Differentiation, T-Lymphocyte; D000954:Antigens, Surface; D004273:DNA, Neoplasm; D011961:Receptors, Fc; D017452:Receptors, IgG; D011971:Receptors, Immunologic