Hepatic veno-occlusive disease-immunodeficiency syndrome
Engelsk navn: Hepatic veno-occlusive disease-immunodeficiency syndrome
Engelske synonym: VODI syndrome
Definisjon
A rare syndrome with combined immunodeficiency characterized by the association of severe hypogammaglobulinemia, combined T and B cell immunodeficiency, absent lymph node germinal centers, absent tissue plasma cells and hepatic veno-occlusive disease.
Fra Orphanet
ORPHA: 79124
Klassifiseringsnivå: Disorder
ICD-10: K76.5