Childhood macrophage activation syndrome differs from infection-associated hemophagocytosis syndrome in etiology and outcome in Taiwan
Hsin-Hsu Chen, Ho-Chang Kuo, Ling Wang, Hong-Ren Yu, Jiun-Min Shen, Kao-Pin Hwang, Kuender D. Yang
Divisions of Pediatric Allergy, Immunology and Rheumatology, Hematology, and Infectious Diseases, Chang Gung Memorial Hospital-Kaohsiung Medical Center; and Chang Gung University, Kaohsiung, Taiwan
Received: August 10, 2006 Revised: August 14, 2006 Accepted: August 18, 2006
Background and purpose:
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome composed of macrophage activation syndrome (MAS), infection-associated hemophagocytosis syndrome (IAHS), malignancy-associated HLH and genetic HLH. Differentiation of MAS from IAHS and other HLH is important for early appropriate treatment.
A retrospective analysis was used to differentiate childhood MAS from IAHS and other HLH in Chang Gung Memorial Hospital (CGMH), Kaohsiung. All relevant clinical features, laboratory data, treatments and outcomes were analysed.
Seventeen patients with childhood HLH were found at CGMH, Kaohsiung in the past decade, and could be classified into 3 categories: IAHS (9 patients), MAS (5 patients), and HLH of unknown etiology (3 patients). The diagnosis of MAS first appeared in this hospital in 2001. Patients with IAHS tended to be younger than those with MAS. Boys were more frequently found in the IAHS group whereas girls (with systemic lupus erythematosus or juvenile idiopathic arthritis) were more frequently found in the MAS group. The majority of mortality cases were noted in the IAHS group (44%, 4/9). All patients with MAS survived with early cyclosporine A treatment.
Childhood MAS is different from IAHS in terms of age, gender, etiology and mortality. Early administration of cyclosporine A for MAS results in a lower mortality. Further prospective studies are required to confirm these findings.
Cyclosporine; Lymphohistiocytosis, hemophagocytic; Macrophage activation; Phagocytosis
J Microbiol Immunol Infect. 2007;40:265-271.