Childhood macrophage activation syndrome differs from infection-associated hemophagocytosis syndrome in etiology and outcome in Taiwan.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Chen
Hsin Hsu
HH
Division of Pediatric Allergy, Immunology and Rheumatology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University, Kaohsiung, Taiwan.
Kuo
Ho Chang
HC
Wang
Ling
L
Yu
Hong Ren
HR
Shen
Jiun Min
JM
Kwang
Kao Pin
KP
Yang
Kuender D
KD
D000293:Adolescent; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine; D005260:Female; D004854:Herpesvirus 4, Human / Q000276:immunology / Q000302:isolation & purification; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology*; D008262:Macrophage Activation; D008297:Male; D016019:Survival Analysis; D013577:Syndrome; D013624:Taiwan / Q000453:epidemiology
D016572:Cyclosporine