Humans ; Macrophage Activation Syndrome

Humans ; Macrophage Activation Syndrome ; diagnosis

Child ; Humans ; Macrophage Activation Syndrome ; diagnosis

Humans ; Macrophage Activation Syndrome ; genetics ; therapy

Infant, Newborn ; Humans ; Macrophage Activation Syndrome/diagnosis*

Humans ; Macrophage Activation Syndrome ; etiology ; Rheumatic Diseases ; complications

Arthritis, Juvenile ; complications ; Child ; Female ; Humans ; Macrophage Activation Syndrome ; complications

Arthritis, Juvenile ; therapy ; Child ; Humans ; Macrophage Activation Syndrome ; therapy ; Plasmapheresis

Arthritis, Juvenile ; complications ; immunology ; Child ; Humans ; Macrophage Activation ; Male ; Syndrome

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