Secondary hemophagocytosis in 3 patients with organic acidemia involving propionate metabolism.
Pediatr Hematol Oncol
; 29(1): 92-8, 2012 Feb.
Article
en En
| MEDLINE
| ID: mdl-21970506
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) may develop secondary to infections, malignancies, immune deficiency syndromes, and rheumatologic and metabolic disorders. Associations between HLH and inborn errors of metabolism, including lysinuric protein intolerance, multiple sulfatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis, have previously been reported in the literature. In this report the authors present 3 children with disorders of propionate metabolism--1 with methylmalonic acidemia and 2 with propionic acidemia--who developed secondary HLH during their metabolic attacks. All patients fulfilled the 5 HLH criteria of the Histiocyte Society. Familial HLH was ruled out by molecular analysis. Plasma exchange was performed for 2 of them. Unfortunately 1 died of multiorgan failure despite intensive therapy. This is the first report of such an association.
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Linfohistiocitosis Hemofagocítica
/
Acidemia Propiónica
/
Errores Innatos del Metabolismo de los Aminoácidos
Tipo de estudio:
Etiology_studies
Límite:
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Hematol Oncol
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2012
Tipo del documento:
Article
País de afiliación:
Turquía