Hepatic venoocclusive disease with immunodeficiency syndrome (VODI) is a primary immunodeficiency characterized by severe hypogammaglobulinemia, combined T and B cell immunodeficiency, absent lymph node germinal centers, and absent tissue plasma cells. The condition has an early onset and it leads to hepatomegaly and liver failure. VODI is caused by homozygous mutation in the SP110 gene.
Mellis and Bale (1976) reported three families with five infants with venoocclusive disease with immunodeficiency. In two of these families, the parents were Lebanese and consangunieus. In the third family, the father was Lebanese.