/hepatic veno-occlusive disease-immunodeficiency syndrome

hepatic veno-occlusive disease-immunodeficiency syndrome

Rare Disease

MONDO:0009338

Also Known As

VODI syndromeVODIfamilial veno-occlusive disease with immunodeficiencyhepatic veno-occlusive disease with immunodeficiencyhepatic venoocclusive disease with immunodeficiencyveno-occlusive disease and immunodeficiency syndrome

Definition

Hepatic veno-occlusive disease-immunodeficiency syndrome is 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.