Clinical characteristics of hemophagocytic lymphohistiocytosis following Kawasaki disease: differentiation from recurrent Kawasaki disease
Blood Research
; : 254-257, 2013.
Article
在 En
| WPRIM
| ID: wpr-25186
Responsible library:
WPRO
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.Key words
全文:
1
索引:
WPRIM
主要主题:
Immunoglobulin G
/
Survival Rate
/
Retrospective Studies
/
Follow-Up Studies
/
Early Diagnosis
/
Diagnosis
/
Drug Therapy
/
Lymphohistiocytosis, Hemophagocytic
/
Lost to Follow-Up
/
Fever
研究类型:
Diagnostic_studies
/
Guideline
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
/
Screening_studies
限制:
Child
/
Female
/
Humans
/
Male
语言:
En
期刊:
Blood Research
年:
2013
类型:
Article