| Dianzani auto-immune lymfoproliferatieve ziekte (aandoening) | | Dianzani auto-immune lymfoproliferatieve ziekte | | DALD
Dianzani autoimmune lymphoproliferative disease
| | Dianzani autoimmune lymphoproliferative disease | | DALD - Dianzani autoimmune lymphoproliferative disease
| | A very rare disorder with characteristics of autoimmunity, lymphadenopathy and/or splenomegaly. The prevalence is not known. The disorder has been reported in fewer than 30 patients to date. Age of onset is highly variable, ranging from childhood to young adulthood. A possible increased risk of cancer has been suggested in these patients. The cause is not known but it is thought to be hereditary. Biologically, DALD has characteristics of normal double-negative T-cells (DNTs) and defective in vitro FAS-mediated apoptosis. The pattern of inheritance of DALD is not known. |
| | Id | 721093000 | | 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 | 275523 |
| SNOMED CT to ICD-10 extended map | | Target | D47.9 | | Rule | TRUE | | Advice | ALWAYS D47.9 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE | | Correlation | SNOMED CT source code to target map code correlation not specified |
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