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hereditaire acute myeloïde leukemie (aandoening)
hereditaire acute myeloïde leukemie
familiaire acute myeloïde leukemie
erfelijke AML
Inherited acute myeloid leukemia
Pure familial acute myeloid leukemia
A rare malignant hematologic disease characterized by clonal proliferation of myeloid blasts, primarily involving the bone marrow, in association with congenital disorders (e.g. Fanconi anemia, dyskeratosis congenita, Bloom syndrome, Down syndrome, congenital neutropenia, neurofibromatosis) and genetic defects predisposing to acute myeloid leukemia. Patients present with signs and symptoms related to ineffective hematopoesis (fatigue, bleeding and bruising, recurrent infections, bone pain) and/or extramedullary site involvement (gingivitis, splenomegaly). Depending on the underlying genetic defect, there may be additional cancer risks and other health problems present.
Id764940002
StatusDefined
Associated withcongenitale ziekte
Associated morphologyacute myeloïde leukemie
Finding sitestructuur van beenmerg
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetC92.0
TermAcute myeloblastenleukemie [AML]
SNOMED CT to Orphanet simple map319465
SNOMED CT to ICD-10 extended map
TargetC92.0
RuleTRUE
AdviceALWAYS C92.0 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified