Division of Haematology and Oncology, Department of Paediatrics, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec.
Allergy Asthma Clin Immunol. 2005 Dec 15;1(4):142-60. doi: 10.1186/1710-1492-1-4-142.
Pediatric hemophagocytic syndrome (HS) is a severe and often fatal clinical disorder. This syndrome is frequently unrecognized, and thus, affected children may receive suboptimal management, leading to an increase in mortality. The purpose of this review is to provide a clinical guide to (1) the recognition of HS based on clinical, biologic, and pathologic features; (2) the identification of the primary cause of HS in a given affected child; and (3) the initiation of effective treatment in a timely manner.
儿童噬血细胞综合征(HS)是一种严重且常致命的临床疾病。这种综合征经常未被识别,因此,受影响的儿童可能接受不理想的治疗,导致死亡率增加。本综述的目的是提供临床指导,以(1)根据临床、生物学和病理学特征识别 HS;(2)确定特定受影响儿童 HS 的原发性原因;和(3)及时开始有效的治疗。