CASE REPORT
Klinefelter's syndrome associated with systemic lupus erythematosus and autoimmune hepatitis
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Nobuhito Sasaki1, Kohei Yamauchi1, Ryo Sato2, Tomoyuki Masuda3, Takashi Sawai3 and Hiroshi Inoue1
| (1) |
Third Department of Internal Medicine, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka 020-8505, Japan |
| (2) |
First Department of Internal Medicine, Iwate Medical University School of Medicine, Morioka, Japan |
| (3) |
Department of Pathology, Iwate Medical University School of Medicine, Morioka, Japan |
Received: 16 March 2006 Accepted: 12 June 2006
Abstract Klinefelter's syndrome (KFS) tends to be associated with immunological disorders. We describe a 37-year-old man who presented signs of testicular atrophy and decreased body hair. He showed pancytopenia and elevated levels of liver enzymes. Chromosome analysis revealed 47XXY karyotype; therefore, he was diagnosed with KFS, with systemic lupus erythematosus and autoimmune hepatitis. Treatment with a high dose of methylprednisolone and methyltestosterone improved thrombocytopenia and symptoms, suggesting that methyltestosterone may have a clinical benefit in the treatment of KFS with a low level of testosterone accompanying immunological disorders.
Key words Autoimmune hepatitis - Klinefelter's syndrome - Systemic lupus erythematosus - Testosterone
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