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KMID : 0366220130480040254
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Korean Journal of Hematology
2013 Volume.48 No. 4 p.254 ~ p.257
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Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease
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Kang Hae-Ryong
Kwon Yong-Hoon
Yoo Eun-Sun
Ryu Kyung-Ha
Kim Ji-Yoon
Kim Heung-Sik
Lee Young-Ho
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Abstract
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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 labo-ratory 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 differ-entiate between HLH and the recurrent form of KD.
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KEYWORD
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Hemophagocytic lymphohistiocytosis, Kawasaki disease, Recurrent
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