title: Seropositive polyarthritis and skin manifestations in T-prolymphocytic leukemia/Sezary cell leukemia variant.
abstract: Sezary cell leukemia (SCL) is a rare T cell neoplasia that has been suggested to be a variant of T-prolymphocytic leukemia (T-PLL). Both disorders have an aggressive clinical course, lymphocytosis with characteristic morphology, lymphadenopathy, hepatomegaly, characteristic cytogenetic abnormalities and mature T cell phenotypes. Skin lesions, however, are mainly found in T-PLL. We describe a patient with T-PLL/SCL, who atypically presented with severe seropositive polyarthritis and skin lesions, responding to treatment with human CD52 antibody, CAMPATH-1H and pentostatin. Meningeal leukemia and an assumed myocardial infiltration subsequently developed. Polyarthritis is common in T large granular lymphocyte leukemia and adult T cell lymphoma-leukemia, but both entities could be ruled out in the present case. In rheumatoid arthritis, an expansion of CD4+ and/or CD8+ T lymphocytes is well documented and this phenomenon is believed to be of pathogenetic importance. We speculate that the T cell clone in the present case had special homing properties or cytokine effects resulting in synovitis.
authors.affiliation: Department of Medicine-County Hospital, Halmstad, Sweden.
authors.forename: A
authors.initials: A
authors.lastname: Dybjer
authors.forename: L
authors.initials: L
authors.lastname: Hellquist
authors.forename: B
authors.initials: B
authors.lastname: Johansson
authors.forename: L
authors.initials: L
authors.lastname: Rydgren
authors.forename: R
authors.initials: R
authors.lastname: Billström
mesh_terms: D001168:Arthritis / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D017809:Fatal Outcome; D006801:Humans; D015463:Leukemia, Prolymphocytic / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D015458:Leukemia, T-Cell / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012751:Sezary Syndrome / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*