Two Cases of Thrombotic Thrombocytopenic Purpura in Systemic Lupus Erythematosus

Kim, Hye-Young; Kim, Hyung-Hoi; Kim, Su-Yung

doi:2007.11.2.288

J Korean Soc Pediatr Nephrol > Volume 11(2); 2007 > Article

J Korean Soc Pediatr Nephrol 2007;11(2): 288-293. doi: https://doi.org/10.3339/jkspn.2007.11.2.288

전신성 홍반성 루프스와 동시 발병한 혈전성 혈소판 감소성 자반증 2예

김혜영, 김형희, 김수영

¹부산대학교 의과대학 소아청소년과
²부산대학교 의과대학 진단검사의학과
³부산대학교 의과대학 소아청소년과

Two Cases of Thrombotic Thrombocytopenic Purpura in Systemic Lupus Erythematosus

Hye-Young Kim, Hyung-Hoi Kim, Su-Yung Kim

¹Departments of Pediatrics, College of Medicine Busan National University
²Departments of Pediatrics, Laboratory Medicine, Busan National University
³Departments of Pediatrics, College of Medicine Busan National University

ABSTRACT

Thrombotic thrombocytopenic purpura(TTP) is a rare but life-threatening multi-system disorder characterized by the classic pentad of clinical features that includes fever, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities and renal dysfunction. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). While it is important to distinguish between the two diseases of therapeutic implication, cases of concurrent TTP and SLE help to elucidate the pathophysiology that underlies each condition. We describe two adolescents with synchronous TTP and SLE, and review the literature.

Key words: Thrombotic thrombocytopenic purpura | Systemic lupus erythematosus | Plasmapheresis