不完全性川崎病合并嗜血细胞综合征1例临床报道并文献复习

Incomplete Kawasaki Disease Complicated with Hemophilic Syndrome: A Case Report and Literature Review

摘要: 目的：了解不完全性川崎病合并嗜血细胞综合征的发生发展过程，提高对该疾病的认识和警惕。方法：回顾分析1例婴幼儿免疫球蛋白治疗无反应型不完全性川崎病合并嗜血细胞综合征的临床资料。结果：该2岁大男性患儿入院时符合不完全川崎病诊断，两次免疫球蛋白冲击治疗无效，仍反复高热，最后发展为嗜血细胞综合征，出现血常规提示3系下降、肝功能异常、血清铁蛋白升高等，骨髓细胞学检测可见噬血现象等，最后按HLH-04化疗方案治疗好转出院。结论：不完全性川崎病免疫球蛋白治疗无效，应早期激素或免疫抑制剂治疗，并提高对该病的早期识别及干预，早期阻断该病发展为嗜血细胞综合征。

Abstract: Objective: To understand the occurrence and development of incomplete Kawasaki disease with the hemophagocytic syndrome, and to raise awareness and vigilance of the disease. Method: The clinical data of incomplete Kawasaki disease with the hemophagocytic syndrome in a 2-year-old boy was retrospectively analyzed. Results: We report here a case of a 2-year-old boy with incomplete Kawa-saki disease which resistant to two doses of immunoglobulins and have recurrent hyperthermia. Eventually develop into hemophagocytic syndrome, in which blood tests demonstrated blood rou-tine showed the decline of 3 lines, abnormal liver function, elevated serum ferritin, and hemopha-gocytosis features on bone marrow aspirate. Finally, recovery was obtained with the HLH-04 chem-otherapy regimen. Conclusion: When the immunoglobulin therapy for incomplete Kawasaki disease is ineffective, early glucocorticoid or immunosuppressive therapy should be used. Besides, we should improve the early recognition and intervention of the disease, and block the development of the disease into hemophagocytic syndrome.