Recurrence of Primary Sclerosing Cholangitis After Living Donor Liver Transplantation

Sumihito Tamura; Yasuhiko Sugawara; Junichi Kaneko; Yuichi Matsui; Junichi Togashi; Masatoshi Makuuchi

Disclosures

Liver International. 2007;27(1):86-94. 

In This Article

Results

Demographics, condition, and treatment before transplantation of the nine patients who underwent LDLT for PSC are summarized in Table 1 and Table 2 . The mean age was 33 years (range 19-50 years). The average model for end-stage liver disease score was 22 (range 16-30). Four (44%) were complicated by inflammatory bowel disease necessitating additional medication. In one patient, cyclosporine was administered for psoriasis. Three patients had variceal bleeding requiring multiple therapies including surgery before liver transplantation. Ursodeoxycholic acid was administered in eight patients. A biliary drainage procedure was necessary in two (Cases 2 and 9), followed by pylorus-preserving pancreaticoduodenectomy in one for suspected lower bile duct carcinoma, which was later ruled out by histologic studies. Case 6 had undergone DDLT in the United States 13 years before, and was admitted for recurrent PSC necessitating retransplantation.

In all but one case, the hemiliver graft was obtained from a biologically related living donor. In one case, a patient listed for DDLT underwent timely LDLT due to her rapidly deteriorating condition using a hemiliver graft obtained from an LDLT recipient who underwent auxiliary partial liver transplantation for familial amyloidotic polyneuropathy (Case 8). The mean age of the live donors was 44 years (range 19-60-years-old; four males, five females). There were no ABO blood-type incompatibilities. Left hemiliver grafts, either with or without the caudate lobe, were obtained in 6 (67%) cases (Cases 1, 2, 5, 7-9). A right hemiliver graft was procured in two cases (Cases 3, 6), and a right lateral sector graft in one (Case 4). The mean ratio of graft weight to recipient standard liver volume was 43% (range 31-53%). None of the live donors required transfusion of packed red blood cells either intra- or postoperatively. There was no mortality among donors.

A summary of the postLDLT course and outcome is depicted in Table 3 and Fig. 1. The median follow-up period was 3.5 years. Overall 5-year patient and graft survival rates were 90% and 71%, respectively (Fig. 1A). Two patients died (Cases 1 and 6). Case 1 died of sepsis caused by bowel perforation due to intestinal lymphoma. The patient had undergone DDLT in the United States after having recurrent PSC in the grafted liver from the initial LDLT. Another patient died of complications related to late-onset hepatic artery thrombosis after LDLT for recurrent PSC following DDLT. In Case 6, DDLT was performed earlier in the United States, complicated by liver abscess, necessitating two liver transplant procedures. Including the above-described patient, recurrent PSC was diagnosed in four (44%) patients ( Table 3 ).

Survival and disease recurrence. (A) Overall patient survival (solid line) and graft survival (dotted line). (B) Freedom from recurrent PSC diagnosed by radiological imaging.

Three patients are alive with recurrent PSC diagnosed by either magnetic resonance cholangiopancreatography or drip infusion cholangiography with multidetector-row helical computer-assisted tomography (Fig. 2B-D). Ratios of freedom from recurrent PSC at 1, 3, and 5 years were 100%, 73%, and 49%, respectively (Fig. 1B). The mean length of time to the diagnosis of recurrent PSC from LDLT was 3.3 years (range 1.1-5.4 years). Cytomegalovirus infection occurred in two patients (Cases 1 and 2). Acute cellular rejection occurred in three (Cases 2, 4, 5) and was successfully treated with steroid recycling. No hepatic artery complications were detected during hospitalization following LDLT. A combination of tacrolimus and corticosteroid was used as standard immunosuppression therapy, except in two patients. Cyclosporine instead of tacrolimus was chosen for one patient (Case 6) to maintain continuity from the previous DDLT immunosuppression regimen. Another patient (Case 9) underwent conversion from tacrolimus to cyclosporine because of uncontrollable de novo diabetes mellitus 2 months after LDLT. None of the patients were tapered off steroids. To date, none have had exacerbation of inflammatory bowel disease necessitating proctocolectomy. There has been no de novo occurrence of inflammatory bowel disease.

Radiologic images of recurrent PSC. (A) MRCP of Case 1. (B) DIC-CT of Case 2. (C) DIC-CT of Case 3. The stent tube from the transjugular intrahepatic portosystemic shunt procedure before transplantation and portal vein dilatation after transplantation are visualized. (D) MRCP of Case 9. DIC-CT, drip infusion cholangiography with multidetector-row helical computer-assisted tomography; MRCP, magnetic resonance cholangiopancreatography.

HLA haplotypes of both the donor and recipient are shown in Table 4 . None of the patients presented with four HLA class I mismatches, including the case of the nonrelated familial amyloidotic polyneuropathy donor (Case 8). At least one HLA-A match and one HLA-B match were recognized in all nine cases. There were two HLA-DR mismatches in two cases (Cases 4 and 8), and one mismatch in six cases (Cases 1-3, 5, 6, 9). None of the patients presented with the HLA-B8DR3 haplotype.

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