We read with great interest the recently updated AASLD Practice Guidelines published in the March issue of Hepatology titled “Evaluation for Liver Transplantation in Adults.” We commend Dr. Martin and colleagues for undertaking an extensive review of the literature. We think it important for your readers to be aware that, among the indications for liver transplant, other rare disorders should be included. Specifically, we write about growing interest in and a growing body of literature indicating that liver transplantation is curative for severe, unremitting acute intermittent porphyria (AIP).[2, 3] This was first described in 2004 and, thus far, most cases have been done in Europe. Recently, the first liver transplantation for AIP in the United States was performed with excellent success and prompt cure at Mt. Sinai Medical Center, NYC (Liu et al., pers. commun.).
It is also worth reminding readers that erythropoietic protoporphyria (EPP) with pigmentary cirrhosis is a well-established indication for liver or combined liver/bone marrow transplantation. Numerous case reports and publications in various peer-reviewed journals attest to the role of liver or combined liver/bone marrow transplantation in patients with chronic liver disease secondary to EPP.[5, 6] In fact, among the porphyrias, chronic liver disease in EPP is probably the best established indication for liver transplant.
We recently published a review article assessing the role of liver transplantation in porphyria. Because of the growing influence of AASLD guidelines for clinical practice and decisions regarding reimbursement, etc., we hope that the AASLD guidelines will soon be amended to take into account liver transplantation for carefully selected patients with porphyrias.