Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease.
Allergy Asthma Proc
; 30(4): 458-62, 2009.
Article
en En
| MEDLINE
| ID: mdl-19772767
ABSTRACT
X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency affecting approximately 1 to 3 per million live male births. Patients are generally healthy until facing a viral infection such as Epstein-Barr Virus and then may develop fulminant infectious mononucleosis and die. XLP patients are also at increased risk of hemophagocytic lymphohistiocytosis (HLH), which may be triggered by assorted viruses. Here we report a novel case of HLH in a patient with XLP. Significant to his presentation is a paradoxical increase in natural killer (NK) cell activity. We hypothesize that this indicates that Parvovirus B19 activates NK cells via a signaling lymphocytic activation molecule-associated protein (SAP)-independent mechanism. Our case demonstrates an important etiology to consider in the differential diagnosis of XLP patients with nonfocal findings and febrile illnesses.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Infecciones Bacterianas
/
Parvovirus B19 Humano
/
Infecciones por Parvoviridae
/
Linfohistiocitosis Hemofagocítica
/
Trastornos Linfoproliferativos
Tipo de estudio:
Etiology_studies
Límite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Allergy Asthma Proc
Asunto de la revista:
ALERGIA E IMUNOLOGIA
Año:
2009
Tipo del documento:
Article
País de afiliación:
Estados Unidos