감염관련 혈구탐식증후군 (Infection-Associated Hemophagocytic Syndrome)으로 추정되는 2예 보고 및 국내 증례 분석

Abstract

The hemophagocytic syndrome is a disorder caused by systemic proliferation of benign histiocytes with avid phagocytosis of blood cells. Although this is an established disease entity, early clinical diagnosis is often difficult. The disease is known to be rare. The clinical and laboratory characteristics of 33 patients with the infection-associated hemophagocytic syndrome were reviewed which included previously reported 31 cases from the literature, and 2 recent cases presented in this study. The patients were 22 males and 11 females with a mean age of 20.8 years(range 1 to 69 years). Ten patients were associated with viral infection and seven with bacterial infection. The infection usually occurs in patients with preexisting immunological abnormalities, but in this study underlying illness was found in only 8 out of the 33 cases. The overall mortality rate was 63.6%, which is a higher percentage than in other countries. Since the clinical course can be fulminant, accurate diagnosis and effective treatment are needed.