Donor Hemosiderosis Does Not Affect Liver Function and Regeneration in the Setting of Living Donor Liver Transplantation

Abstract

Living donor liver transplantation (LDLT) demands a careful assessment of abnormal findings discovered during the evaluation process to determine if there will be any potential risks to the donor or recipient. Varying degrees of elevated hepatic iron levels are not uncommonly seen in otherwise healthy individuals. We questioned whether mild expression of hemosiderin deposition presents a safety concern when considering outcomes of living donation for both the donor and the recipient. We report on three LDLT patients who were found to have low-to moderate-grade hemosiderin deposition on liver biopsy. All other aspects of their evaluation proved satisfactory, and the decision was made to proceed with donation. There were no significant complications in the donors, and all demonstrated complete normalization of liver function postoperatively, with appropriate parenchymal regeneration. The recipients also had unremarkable postoperative recovery. We conclude that these individuals can be considered as potential donors after careful evaluation.

Genetic, hematological, and immunological disorders transmissible with liver transplantation

“…However, 2 of the 3 donors tested negative for HH mutations whereas the last was a compound heterozygote. Thus, liver allografts from donors with known HH should be considered carefully as marginal livers given the likely presence of sideronecrosis and mild chronic inflammation with fibrosis on donor biopsy . The liver from a young donor with HH who had a normal liver biopsy could be considered for transplantation.…”

“…Multiple case reports have described donor-derived transmission of genetic cholestatic disorders, urea cycle disorders, glucose-6phosphate deficiency (G6PD), maple syrup urine disease (MSUD), primary hyperoxaluria (PH), hereditary hemochromatosis (HH), CF, PCLD, acute intermittent porphyria (AIP), FAP, and pseudoxanthoma elasticum (PXE), with LT. Penetrance and time to symptoms may vary from a few months to years depending on the disease. (4)(5)(6)(7)(8)(9) Numerous heritable metabolic diseases can be transmitted with LT. In the case of many autosomal recessive disorders, affected persons are recognized and thus screened out as potential organ donors.…”

“…However, both the donor and recipient developed severe hyperammonemia postoperatively, likely under the stress of LT. Urine samples from both demonstrated high levels of orotic acid confirming the diagnosis of OTCD. The recipient then deteriorated neurologically and Genetic cholestatic disorders GS (18)(19)(20) DJS (21) Thrombotic diseases AT deficiency (10) Protein C deficiency (11) Factor V Leiden (13,14) Protein S deficiency (15) ITP (132)(133)(134)(135)(136) Urea cycle disorders OTCD (4,5) Bleeding disorders Hemophilia A (123) Hemophilia B (124,125) Factor VII deficiency (122) Factor XI deficiency (126,(128)(129)(130) Food allergies (138,(140)(141)(142) Maple syrup urine disease (24,25,67,69) PH (29)(30)(31)(32)70) HH (6)(7)(8) CF (34) A1AT deficiency (35,36,79) PCLD (40)…”

“…Maple syrup urine disease Likely safe, extrapolated from domino LT (24,25,67,69) Likely safe, extrapolated from domino LT (24,25,67,69) Likely safe (26)(27)(28) PH Not recommended, extrapolated from domino LT (29)(30)(31)(32)70) Not recommended, extrapolated from domino LT (29)(30)(31)(32)70) No available data HH Likely safe (6,7,33) Likely safe (8) No available data CF Likely safe (34) No available data, but likely safe as in DDLT…”

“…These complications include the development or refractoriness of hyperammonemia, mental status changes, and lactic acidosis in the short term, significant hyperferritinemia or hyperlipidemia, or the development of de novo hypercoaguability or a bleeding diathesis. (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15) Such transmissions can be categorized in several ways: first, whether the transplant was performed using a live or deceased donor without pre-LT recognition of a disorder (ie, occult heterozygous urea cycle defects); and second, with a known pre-LT transmissible condition (cystic fibrosis [CF]) or a domino transplant (ie, familial amyloidotic polyneuropathy [FAP]). Finally, whether the donor had a known (ie, polycystic liver disease [PCLD]) or unrecognized forme fruste (ie, Ehlers-Danlos, alpha-1-antitrypsin [A1AT]) of heterozygosity for a transmissible disease.…”