Clinical characteristics of hemophagocytic lymphohistiocytosis following kawasaki disease: Differentiation from recurrent kawasaki disease

Hae Ryong Kang, Yong hoon Kwon, Eun Sun Yoo, Kyung Ha Ryu, Ji Yoon Kim, Heung Sik Kim, Hwang Min Kim, Young Ho Lee

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

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.

Original languageEnglish
Pages (from-to)254-257
Number of pages4
JournalBlood Research
Volume48
Issue number4
DOIs
StatePublished - Dec 2013

Keywords

  • Hemophagocytic lymphohistiocytosis
  • Kawasaki disease
  • Recurrent

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