Pathological classification of porphyria
Hepatic porphyrias:
- acute intermittent porphyria (AIP)
- variegate porphyria (VP)
- hereditary coproporphyria (HCP)
- aminolevulinic acid dehydratase porphyria (ADP), of which only six definite cases have been reported
- porphyria cutanea tarda (PCT)
Erythropoietic porphyrias:
- erythropoietic porphyria (EP)
- erythropoietic protoporphyria (EPP)
- erythropoietic coproporphyria (ECP)
Hepatic / erythropoietic:
- hepatoerythropoietic porphyria (HEP)
In hepatic porphyrias there is excess hepatic production of porphobilinogen. In erythropoietic porphyrias there is increased production of porphyrins in red blood cells due to enzyme deficiency.
Reference:
- Karim Z, Lyoumi S, Nicolas G, et al; Porphyrias: A 2015 update. Clin Res Hepatol Gastroenterol. 2015 Jul 1. pii: S2210-7401(15)00120-5.
- Edel Y, Mamet R. Porphyria: What Is It and Who Should Be Evaluated?. Rambam Maimonides Med J. 2018;9(2):e0013. Published 2018 Apr 19. doi:10.5041/RMMJ.10333
Related pages
Create an account to add page annotations
Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.