title: Myositis in a patient with large granular leukocyte leukemia.
abstract: We describe the case of a 58-year-old patient with subacute progressive weakness in both legs accompanied by recurrent opportunistic infections. White cell count was normal, but immunophenotyping revealed an increased number of CD8(+) T cells and deficiency of natural killer cells, B cells, and CD4(+) T cells in the peripheral blood. Large granular leukocyte (LGL) leukemia was diagnosed based on a clonal T-cell receptor rearrangement. Muscle biopsy demonstrated severe myositis with extensive CD8(+) T-cell infiltrates. Since no evidence of microbial muscle infection was found, autoimmune myositis was diagnosed. Immunosuppressive treatment resulted in clinical improvement and normalization of creatine kinase (CK) serum levels. The immunological phenotype of the patient and the positive response to treatment adds further to the concept that CD8(+) T cells mediate disease in autoimmune myositis.
authors.affiliation: Clinical Neuroimmunology Group, Department of Neurology, Philipps University, Rudolf Bultmann Strasse 8, Marburg 35033, Germany.
authors.forename: Berit
authors.initials: B
authors.lastname: Rosche
authors.forename: Marc
authors.initials: M
authors.lastname: Jacobsen
authors.forename: Sabine
authors.initials: S
authors.lastname: Cepok
authors.forename: Peter
authors.initials: P
authors.lastname: Barth
authors.forename: Norbert
authors.initials: N
authors.lastname: Sommer
authors.forename: Bernhard
authors.initials: B
authors.lastname: Hemmer
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000473:pathology; D006801:Humans; D015458:Leukemia, T-Cell / Q000150:complications* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000150:complications / Q000473:pathology; D009220:Myositis / Q000150:complications* / Q000473:pathology*; D009431:Neural Conduction