Hepatic veno-occlusive disease-immunodeficiency syndrome

Orpha code: 79124OMIM code: 235550

Definition

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.

Disease data
Classification

Disease

Synonyms
VODI syndrome
Zespół VODI
ORPHA code
79124
OMIM code
235550
ICD10 code
K76.5
ICD11 code
4A01.33

No additional description.

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