Beffermann C Nicole, Pilcante S Javier, Ocqueteau T Mauricio, Sarmiento M Mauricio
Rev Med Chil. 2015 Sep;143(9):1172-8. doi: 10.4067/S0034-98872015000900010.
Hemophagocytic syndrome is a severe condition of excessive immune activation that has a high mortality in the absence of treatment. The syndrome is classified as primary if associated with congenital or hereditary problems, or secondary/acquired if associated with infectious, autoimmune or oncology diseases. We report four adult cases of the syndrome, one with viral, two with autoimmune and one with idiopathic causes who were successfully treated with HLH 94-04 chemotherapy protocol. Our experience shows that a high index of suspicion, early diagnosis and an opportune therapy are essential in the treatment of this disease.
噬血细胞综合征是一种严重的过度免疫激活状态，若不治疗死亡率很高。如果与先天性或遗传性问题相关，该综合征被分类为原发性；如果与感染性、自身免疫性或肿瘤性疾病相关，则为继发性/获得性。我们报告了4例该综合征的成年病例，其中1例由病毒引起，2例由自身免疫引起，1例病因不明，均采用HLH 94 - 04化疗方案成功治疗。我们的经验表明，高度的怀疑指数、早期诊断和适时的治疗对该病的治疗至关重要。
