Blood Res.  2013 Dec;48(4):254-257. 10.5045/br.2013.48.4.254.

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

• Kang HR
• Kwon YH
• Yoo ES
• Ryu KH
• Kim JY
• Kim HS
• Kim HM
• Lee YH

• KMID: 2172872
• DOI: http://doi.org/10.5045/br.2013.48.4.254

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.

Keyword

Hemophagocytic lymphohistiocytosis; Kawasaki disease; Recurrent

MeSH Terms

Child
Diagnosis
Drug Therapy
Early Diagnosis
Female
Fever
Follow-Up Studies
Humans
Immunoglobulin G
Lost to Follow-Up
Lymphohistiocytosis, Hemophagocytic*
Male
Mucocutaneous Lymph Node Syndrome*
Retrospective Studies
Survival Rate
Immunoglobulin G

Figure

• 

  Fig. 1 Cumulative survival rate of patients with hemophagocyticlymphohistiocytosis following Kawasaki disease.

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