Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease.
- Author:
Hae Ryong KANG
1
;
Yong Hoon KWON
;
Eun Sun YOO
;
Kyung Ha RYU
;
Ji Yoon KIM
;
Heung Sik KIM
;
Hwang Min KIM
;
Young Ho LEE
Author Information
- Publication Type:Original Article
- Keywords: Hemophagocytic lymphohistiocytosis; Kawasaki disease; Recurrent
- MeSH: 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
- From:Blood Research 2013;48(4):254-257
- CountryRepublic of Korea
- Language:English
- 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.
