Kawasaki Disease Complicated With Macrophage Activation Syndrome: A Systematic Review.
J Pediatr Hematol Oncol
; 39(6): 445-451, 2017 08.
Article
em En
| MEDLINE
| ID: mdl-28562511
ABSTRACT
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases. Coronary abnormalities were especially high in this group of patients (46%) and 9 patients died (13%). The persistence of fever with splenomegaly, hyperferritinemia, thrombocytopenia, and elevated aspartate aminotransferase (AST) should prompt the consideration of MAS complicating KD.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Ativação Macrofágica
/
Síndrome de Linfonodos Mucocutâneos
Tipo de estudo:
Systematic_reviews
Limite:
Adolescent
/
Child
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Child, preschool
/
Female
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Humans
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Infant
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Male
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article