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A transitional variant of a T‐lymphoproliferative malignancy
Author(s) -
Jønsson V.,
Wantzin G. Lange,
Badsberg E.,
Menné T.,
Videbæk Aa.
Publication year - 1986
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1986.tb01729.x
Subject(s) - lymphoproliferative disease , malignancy , clone (java method) , pathology , lymphoproliferative disorders , population , malignant transformation , mycosis fungoides , medicine , lymphoproliferative response , disease , immunology , biology , lymphoma , dna , environmental health , genetics , peripheral blood mononuclear cell , biochemistry , in vitro
2 cases of malignant T‐lymphoproliferative disease are reported. The proliferating cell was a large blast expressing E and Fcγ receptors but no helper or suppressor phenotypes and no SmIg. Skin infiltrates were the dominant clinical sign with conspicuous perivascular aggregations of T, E, Fcγ 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, Fcγ lymphocyte and practically no response to cytostatic treatment.