[Macrophage activation syndrome: a diagnostic challenge].
Macrophage activation syndrome, or hemophagocytic syndrome, is a rare disease with high morbidity and mortality. It is a disorder of the mononuclear phagocyte system. Two forms have been described; primary or familial hemophagocytic lymphohistiocytosis and secondary or sporadic hemophagocytic syndrome. Diagnosis of macrophage activation syndrome poses a real challenge for the pediatrician and in many cases is only made at autopsy. We describe two patients with hemophagocytic syndrome associated with parvovirus B19 and Epstein-Barr virus infection.
Soult Rubio
J A
JA
Unidad de Cuidados Intensivos Pediátricos, Hospital Infantil Universitario Virgen del Rocío, Sevilla, Spain. jasoult@terra.es
García Bernabeu
V
V
Sánchez Alvarez
M J
MJ
Muñoz Sáez
M
M
López Castilla
J D
JD
Tovaruela Santos
A
A
D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D005334:Fever; D004854:Herpesvirus 4, Human; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000821:virology*; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology; D010322:Parvoviridae Infections / Q000175:diagnosis* / Q000503:physiopathology; D016732:Parvovirus B19, Human; D013163:Splenomegaly