Autosomal recessive hereditary disorder	Combined immunodeficiency disease	Congenital immunodeficiency disease	Disorder of immune structure	Hereditary cancer-predisposing syndrome	Hereditary disorder of immune system	Immunodeficiency associated with chromosomal abnormality
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Combined immunodeficiency due to OX40 deficiency (disorder)
	Combined immunodeficiency due to OX40 deficiency
	Combined immunodeficiency with childhood-onset Kaposi sarcoma
	A rare combined T and B cell immunodeficiency with characteristics of susceptibility to develop an aggressive, childhood-onset, disseminated, cutaneous and systemic Kaposi sarcoma. There is evidence the disease is caused by homozygous mutation in the OX40 gene (TNFRSF4) on chromosome 1p36.
	gecombineerde immunodeficiëntie door OX40-deficiëntie
	gecombineerde immuundeficiëntie door OX40-deficiëntie gecombineerde immuundeficiëntie met verstoorde immuniteit tegen HHV-8 gecombineerde immuundeficiëntie met verstoorde immuniteit tegen humaan herpesvirus 8 gecombineerde immunodeficiëntie met in kindertijd optredend Kaposi-sarcoom

Id	766879006
Status	Primitive

Finding site	Structure of immune system
Occurrence	Congenital

Due to

Chromosomal disorder

Pathological process

Abnormal immune process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	D81.8
Rule	TRUE
Advice	ALWAYS D81.8
Correlation	SNOMED CT source code to target map code correlation not specified