Clinical characteristics of hemophagocytic lymphohistiocytosis following kawasaki disease: Differentiation from recurrent kawasaki diseaseopen access
- 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
- Issue Date
- Dec-2013
- Publisher
- Korean Society of Hematology
- Keywords
- Hemophagocytic lymphohistiocytosis; Kawasaki disease; Recurrent
- Citation
- Blood Research, v.48, no.4, pp.254 - 257
- Indexed
- SCOPUS
KCI
- Journal Title
- Blood Research
- Volume
- 48
- Number
- 4
- Start Page
- 254
- End Page
- 257
- URI
- https://scholarworks.bwise.kr/hanyang/handle/2021.sw.hanyang/161245
- DOI
- 10.5045/br.2013.48.4.254
- ISSN
- 2287-979X
- Abstract
- Background: 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. Methods: 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. Results: 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. Conclusion: 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.
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