Autosomal recessive hereditary disorder
Disorder of hematopoietic morphology
Malignant neoplasm of lymphoid, hematopoietic and/or related tissue
Uroporphyrinuria
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Erythropoietic uroporphyria associated with myeloid malignancy (disorder)
Erythropoietic uroporphyria associated with myeloid malignancy
A rare porphyria characterized by a pre-existing myeloid disorder, skin fragility and blistering on the exposed areas, and hemorrhagic bullae typically on the back of the hands. Urine, plasma and fecal porphyrins are increased.
Id783615009
StatusPrimitive
Associated morphologyMyelodysplastic neoplasm
SNOMED CT to Orphanet simple map
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetE80.2
TermOverige gespecificeerde vormen van porfyrie
SNOMED CT to ICD-10 extended map
TargetE80.2
RuleTRUE
AdviceALWAYS E80.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified