Autosomal recessive hereditary disorder	Combined immunodeficiency disease	Hereditary cancer-predisposing syndrome	Lymphoproliferative disorder
\|	\|	\|	\|

Autosomal recessive lymphoproliferative disease (disorder)
	Autosomal recessive lymphoproliferative disease
	A rare combined T and B cell immunodeficiency with a predisposition to lymphoproliferative syndrome. It is characterized by persistent symptomatic Epstein-Barr viremia and hypogammaglobulinemia variably presenting with fever, lymphadenopathy and systemic inflammatory conditions including hepatitis, pneumonia and sepsis. It may be associated with lymphoma, hemophagocytic lymphohistiocytosis, and aplastic anemia.
	autosomaal recessieve lymfoproliferatieve ziekte
	autosomaal recessieve lymfoproliferatieve aandoening

Id	771309000
Status	Primitive

Associated morphology

Lymphoproliferative disorder

Pathological process

Abnormal immune process

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	D47.9
Rule	TRUE
Advice	ALWAYS D47.9 \| POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
Correlation	SNOMED CT source code to target map code correlation not specified