Abstract
Dear Editor, Patients with T cell large granular lymphocyte (T-LGL) leukemia have a high prevalence of autoantibodies and associated autoimmune disease. Behcet's disease (BD) is an autoimmune disease, the inflammation of which is thought to be mediated by inflammatory cytokines, including interleukin 18 (IL-18) and tumor necrosis factor α (TNFα; however, concurrent diagnosis of T-LGL leukemia with BD is extremely rare. A 56-year-old Japanese male patient was referred to our hospital in May 2002 for low grade fever and general fatigue. He had a history of recurrent ulcers over the scrotum, inguinal region, and in the mouth. Physical examination showed anemia, ulcers on the scrotum, and cutaneous pustules on the back. Pathergy skin test was positive. Endoscopic examinations of his gastrointestinal tract revealed multiple ulcers in the ileocecal region. Thus, he was diagnosed as having an incomplete form of BD according to the International Study Group criteria. His peripheral blood counts were hemoglobin 8.9 g/dl; platelets, 186×10/l; and WBC, 5.7×10/l with a differential of 78% lymphocytes. These lymphocytes were morphologically LGL. Flow cytometric analysis showed that the peripheral blood lymphocytes predominantly expressed CD3+, CD8+, CD16+, and TCRαβ+. Clonal T cell receptor TCRcβ1 gene rearrangement of peripheral blood mononuclear cells was detected (Fig. 1). Cytogenetic analysis revealed a normal karyotype. C-reactive protein was 4.2 mg/dl. The serum levels of soluble FasL and IL-18 concentration were elevated at 0.32 ng/ml (normal <0.05 ng/ml) and 0.83 ng/ml (normal <0.1 ng/ml), respectively, whereas the TNF-α level was normal. The levels of FasL and IL-18 in the supernatant of peripheral blood T cells were 0.23 ng/ml and 0.48 ng/ml, respectively, whereas these species were not found in the supernatant of peripheral blood non-T cell cultures. Based on these data, we diagnosed our patient to have CD8+ T-LGL leukemia. He has been treated with daily oral prednisolone (5 mg/day) and additional colchicines for BD; the oral and genital ulcers slightly improved after addition of the colchicines. Anemia and neutropenia did not respond to this treatment, but no infectious complications due to neutropenia occurred. LGL leukemia is an atypical chronic lymphoproliferative disease defined by a monoclonal distribution. There are two major types of LGL leukemia, CD3+ LGL and CD3− LGL. The former are cytotoxic T cells (T-LGL) and the latter natural killer cells (NK-LGL) [1]. Clinical autoimmune diseases, mainly rheumatoid arthritis are reported; however, Sjogren's syndrome, systemic lupus erythematosus, or other Ann Hematol (2008) 87:585–586 DOI 10.1007/s00277-008-0438-3
Published Version
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