04093 Portal hypertension in advanced protoporphyric hepatopathy: a case report

demonstrated elevated total red blood cell porphyrin of 36.1 umol/L RBC (reference range <1.8) with normal levels of zinc protoporphyrin 1.3 umol/L RBC (reference range <1.6).Liver function tests were unremarkable.She was mildly anaemic, in keeping with MDS.A diagnosis of erythropoietic protoporphyria was made.She was able to cease prednisolone and antihistamines.We counselled her on the need to avoid sun exposure and implement strict sun protection strategies.Presently, she continues venetoclax monotherapy.A bone marrow transplant would potentially be curative for both MDS and EPP however, she has previously been deemed inappropriate due to her age.While acquired EPP is a rare entity, our case exemplifies the need to screen for EPP in patients of any age where there is clinical suspicion, especially in patients with an underlying haematological disorder.Treatment of the abnormal bone marrow clone with chemotherapy temporarily reduces EPP symptoms.