Hepatic veno-occlusive disease - immunodeficiency syndrome

Description

Hepatic veno-occlusive disease - immunodeficiency syndrome is characterised by the association of severe hypogammaglobulinemia, combined T and B cell immunodeficiency, absent lymph node germinal centres, absent tissue plasma cells and hepatic veno-occlusive disease.

Index terms

Hepatic veno-occlusive disease - immunodeficiency syndrome
VODI - [Veno-occlusive disease immunodeficiency] syndrome

Exclusions from above levels

02 – Neoplasms
18 – Complications of pregnancy, childbirth and the puerperium
20 – Developmental anomalies

Sibling codes

4A01.30 – Immunodeficiency due to defects of the thymus
4A01.31 – DNA repair defects other than combined T-cell or B-cell immunodeficiencies
4A01.32 – Immuno-osseous dysplasia
4A01.34 – Hyperimmunoglobulin E syndromes

Foundation entity

Hepatic veno-occlusive disease - immunodeficiency syndrome – http://id.who.int/icd/entity/712514250

Part of: 4A01.3: Other well-defined immunodeficiency syndromes due to defects in adaptive immunity › 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system