autosomaal recessieve lymfoproliferatieve ziekte (aandoening) | | autosomaal recessieve lymfoproliferatieve ziekte | | autosomaal recessieve lymfoproliferatieve aandoening
| | Autosomal recessive lymphoproliferative disease | | A rare autosomal recessive primary immunodeficiency characterized by Epstein-Barr virus (EBV)-triggered lymphoproliferative disorders such as malignant B-cell proliferation, Hodgkin lymphoma, B-cell lymphoma and EBV-driven hemophagocytic lymphohistiocytosis (HLH). Aplastic anemia and inflammatory disorders such as uveitis and oral ulcers are also observed. |
| 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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