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

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