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autosomaal recessieve lymfoproliferatieve ziekte (aandoening)
autosomaal recessieve lymfoproliferatieve ziekte
autosomaal recessieve lymfoproliferatieve aandoening
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.
Id771309000
StatusPrimitive
Pathological processafwijkend immuunproces
Associated morphologylymfoproliferatieve aandoening
referentieset met complexe 'mapping' naar ICD-10
TargetD47.9
RuleTRUE
AdviceALWAYS D47.9 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified