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J Yeungnam Med Sci > Volume 34(2); 2017 > Article
Yeungnam University Journal of Medicine 2017;34(2):260-264.
DOI:    Published online December 31, 2017.
Budd-Chiari syndrome with antiphospholipid syndrome and systemic lupus erythematosus in a patient with Klinefelter's syndrome
Mingee Lee, Jin Young Huh, Ji Hyang Lee, Sun myoung Kang, Jae Yong Lee, Oh Chan Kwon, Eun Na Kim, Jihun Kim, Danbi Lee
1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.
Key Words: Klinefelter's syndrome, Systemic lupus erythematosus, Antiphospholipid syndrome, Budd-Chiari syndrome, Liver cirrhosis
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