Klinefelter's syndrome associated with systemic lupus erythematosus and autoimmune hepatitis.
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.
Sasaki
Nobuhito
N
Third Department of Internal Medicine, Iwate Medical University School of Medicine, 19-1 Uchimaru, Morioka, 020-8505, Japan.
Yamauchi
Kohei
K
Sato
Ryo
R
Masuda
Tomoyuki
T
Sawai
Takashi
T
Inoue
Hiroshi
H
D000328:Adult; D045930:Anabolic Agents / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000097:blood; D002869:Chromosome Aberrations; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007713:Klinefelter Syndrome / Q000150:complications* / Q000188:drug therapy; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008777:Methyltestosterone / Q000627:therapeutic use
D045930:Anabolic Agents; D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D008777:Methyltestosterone; D008775:Methylprednisolone