Korean J Pediatr Infect Dis.  2010 Dec;17(2):177-181.

Macrophage Activation Syndrome as the Extreme Form of Kawasaki Disease

Affiliations
  • 1Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea. sylee@catholic.ac.kr

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.

Keyword

Macrophage activation syndrome; Hemophagocytic lymphohistiocytosis; Kawasaki disease

MeSH Terms

Bone Marrow
Fever
Humans
Immunoglobulins
Lymphohistiocytosis, Hemophagocytic
Macrophage Activation
Macrophage Activation Syndrome
Macrophages
Mucocutaneous Lymph Node Syndrome
Organic Chemicals
Immunoglobulins
Organic Chemicals
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