| 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. |
| | Id | 771309000 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | D47.9 | | Term | Neoplasma met onzeker of onbekend gedrag van lymfoïd, hematopoëtisch en verwant weefsel, niet gespecificeerd |
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| SNOMED CT to Orphanet simple map | 238505 |
| SNOMED CT to ICD-10 extended map | | 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 |
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