Macrophage Activation Syndrome as the Extreme Form of Kawasaki Disease.
- Author:
Hyoun Jin PARK
1
;
Yoon Jeong CHO
;
E Young BAE
;
Ui Yoon CHOI
;
Soo Young LEE
;
Dae Chul JEONG
;
Kyung Yil LEE
;
Jin Han KANG
Author Information
1. Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea. sylee@catholic.ac.kr
- Publication Type:Case Report
- Keywords:
Macrophage activation syndrome;
Hemophagocytic lymphohistiocytosis;
Kawasaki disease
- MeSH:
Bone Marrow;
Fever;
Humans;
Immunoglobulins;
Lymphohistiocytosis, Hemophagocytic;
Macrophage Activation;
Macrophage Activation Syndrome;
Macrophages;
Mucocutaneous Lymph Node Syndrome;
Organic Chemicals
- From:Korean Journal of Pediatric Infectious Diseases
2010;17(2):177-181
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Few cases of macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) during the acute febrile phase of Kawasaki disease (KD) have been reported. We report on a case of a 19 month-old girl with MAS or reactive HLH during the course of KD. Despite immunoglobulin and steroid therapy, she showed persistent fever with hepatosplenomegaly and evidence of hemophagocytosis in the bone marrow. A high index of suspicion for clinical features associated with MAS is necessary for KD patients in order to provide appropriate treatment.
