Table 1.
Review of orthotopic liver transplant cases in sickle cell disease patients.
| Study citation | Sex | Age | HbType | Liver pathologies | Transfusion management (pre-op goal) | Transfusion management (post-op goal) | Outcome |
|---|---|---|---|---|---|---|---|
| Perini 20109 | M | 37 | HbS beta thalassemia | Hepatitis C; hemosiderosis | Not stated | Post-op target HbS<30% with exchange transfusions | Death after 5 months 3 months: acute sickle hepatic crisis with elevated LFTs and RUQ pain-resolved with exchange transfusions and hydroxycarbamide 5 months: died from intracranial hemorrhage from Moyamoya disease |
| Greenberg 201010 | F | 30 | HbSS | Extensive hepatocyte necrosis but no signs of SC1C or pregnancy related hepatopathy | RBC exchange from presenting HbS=80% to HbS=26% |
Post-op: HbS<30% | Survival POD 1-3:ICU requiring mechanical ventilation POD 28: discharge home |
| Baichi 20056 | F | 27 | HbSS | SC1C; autoimmune hepatitis; cirrhosis | Presented with HbS=69.6%, exchange transfusion to keep HbS<10% | Exchange transfusion to keep HbS<10% | Death on POD 35 from peritoneal bleed and MOF |
| Baichi 20056 | F | 26 | HbSS | Sclerosing cholangitis; periductal fibrosis consistent with intrahepatic cholestasis | Presented initially with HbS=15.1%; exact amount of transfusion not stated | HbS<10% without any transfusions during OLT hospitalization | Death on POD 85 from septic shock and MOF |
| Kindscher 19952 | F | 47 | HbSS | Chronic hepatitis C; cirrhosis | Exchanged 4 units to reduce HbS from 52% to 27% | Target HbS<30% | Survival: discharge home on POD 40; no signs of rejection Complication: cerebral hemorrhage POD 12 managed conservatively w/ CNS improvement after 3 months f/u |
| Ross 200211 | M | 49 | HbSS | SC1C | Initially presented with Hb=5.2 and HbS=52%, which prompted 6 U RBC transfusion | Target HbS<20% | Death: discharge POD 33 Complication: biliary anastomosis leak repair POD 7 22 months Post-op: death from pulmonary embolus; autopsy reveals no cholestasis or rejection |
| Gilli 20025 | M | 22 | HbS beta thalassemia | SC1C; cirrhosis | Target HbS<30% | Target HbS<20% | Survival: mild intrahepatic sickling 3 months post-op; no signs of rejection 2 years post-op |
| Delis 200612 | F | 19 | HbSD | Hepatitis B; cirrhosis | Not stated | Exchange transfusion at 17 months post-op for elevated LFTs: decreased HbS from 33.8 to 7.8% | Survival: no rejection Complication: intrahepatic cholestasis of allograft resolved after transfusion |
| Van den Hazel 200313 | M | 23 | HbSS | Hemochromatosis | 5 U RBCs exchange decreased HbS from 32% to 19% | Intra-op: transfused 44 U RBCs HbS=4.9% 1 wk post-op (w/o tranfusion) | Survival: no rejection; liver functions intact at 5.5 yrs post-OLT |
| Lerut 199914 | F | 42 | HbS beta thalassemia | Cryptogenic cirrhosis | Target not stated | Target HbS<10% | Survival Complication: partial graft infarction/necrosis at post-op 6 month-spontaneous recovery |
| Emre200015 | M | 6 | HbSS | Dilated sinusoids filled with sickled RBCs | HbS=21.6% at presentation prior to first OLT; pre-op target not stated | Target HbS<20% | Death from sepsis after 3rd transplant; 1st graft failed due to veno-occlusive disease; 2nd graft failed from hepatic artery thrombosis |
| Lang 199516 | M | 11 | HbSS | Biliary cirrhosis | Not stated | HbS<20% | Survival; intact graft function |
| Meekel 200717 | N/A | 8,17,17 | Not specified | 3 had intrahepatic cholestasis; 1 had chronic hepatitis C | Exchange transfusions for target HbS<25% and Hb>9 | Exchange transfusions for target HbS<25% and Hb>9 | 2 Survived 1 Death: recurrent intrahepatic cholestasis and (3 patients) chronic graft failure, and died 6 yrs post-op from subdural hematoma from fall |
Hb, hemoglobin; SCIC, sickle cell intrahepatic cholestasis; RBC, red blood cell; OLT, orthotopic liver transplant; MOF, multiorgan failure.