Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease

• Published in: Blood research Vol. 48; no. 4; pp. 254 - 257
• Main Authors: Kang, Hae-Ryong, Kwon, Yong-hoon, Yoo, Eun-Sun, Ryu, Kyung-Ha, Kim, Ji Yoon, Kim, Heung-Sik, Kim, Hwang Min, Lee, Young-Ho
• Format: Journal Article
• Language: English
• Published: Switzerland Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 01.12.2013; 대한혈액학회
• Subjects: Original; 병리학; Hemophagocytic lymphohistiocytosis; Kawasaki disease; Recurrent
• ISSN: 2287-979X; 2288-0011
• DOI: 10.5045/br.2013.48.4.254

Our aim was to investigate the clinical pattern of hemophagocytic lymphohistiocytosis following Kawasaki disease (HLH-KD), to enable differentiation of HLH from recurrent or refractory KD and facilitate early diagnosis. We performed a nationwide retrospective survey and reviewed the clinical characteristics of patients with HLH-KD, including the interval between KD and HLH, clinical and laboratory findings, treatment responses, and outcomes, and compared them with historical data for both diseases. Twelve patients with HLH-KD, including 5 previously reported cases, were recruited. The median age was 6.5 years (range, 9 months-14.7 years). Eight patients were male and 4 were female. The median interval between the first episode of KD and the second visit with recurrent fever was 12 days (3-22 days). Of the 12 children, 2 were initially treated with intravenous IgG (IVIG) for recurrent KD when they presented at the hospital with recurrent fever. Eventually, 10 children received chemotherapy under an HLH protocol and 2 received supportive treatment. Two patients died of combined infections during chemotherapy, 1 was lost to follow up, and 9 remain alive. The overall survival rate at 4 years was 81.1% with a median follow up of 45.1 months. A diagnosis of HLH-KD should be considered when symptoms similar to recurrent KD develop within 1 month of the first episode of KD. Our findings will help physicians differentiate between HLH and the recurrent form of KD.