Emergency ABO-incompatible living donor liver transplantation in Wilson disease-induced acute liver failure.

We report the clinical outcome of an emergency ABO incompatible-liver transplantation (LT) for an 8-year-old child with Wilson's disease-induced acute liver failure. The pretransplant anti-A antibody titer was 1:64, and hence he underwent three cycles of conventional plasma exchange as pretransplant liver supportive treatment for deranged coagulopathy and liver function followed by one cycle of immunoadsorption (IA) prior to LT. The posttransplant immunosuppression consisted of rituximab, tacrolimus, mycophenolate mofetil, and corticosteroid. The patient had anti-A isoagglutinin rebound with elevated aminotransferases levels from postoperative day 7 for which he was restarted on IA plasmapheresis, but antibody titers did not decrease. Hence, he was switched to conventional plasmapheresis (CP) with which anti-A antibody titers decreased. The total dose of rituximab (150 milligrams/square meter of body surface area) was given in two divided doses of 75 mg at D-1 and D + 8 which was much less than the dose conventionally advocated (375 milligrams/square meter of body surface area). He is clinically well with good graft function without rejection after 1 year of follow-up. This case illustrates that IA and CP in conjunction with adequate immunosuppression is a viable approach in emergency ABO-incompatible-LT in Wilson disease-induced acute liver failure.

Patient blood management in a patient with multiple red cell antibodies (anti-C, anti-e, and anti-K) undergoing liver transplant in South India: A team approach.

End-stage liver disease (ESLD) patients undergoing liver transplant (LT) surgery are often multiply alloimmunized and pose significant challenges to the transfusion services in terms of red cell cross-match incompatibility, unpredictable blood requirements, and often lead to significant delays in availing compatible red cell units. We report a case of a 64-year-old female from Bahrain, a known case of hepatitis C-related ESLD referred for LT surgery. She had a history of multiple uneventful transfusions in the preceding year. Her blood group was A-positive, direct antiglobulin test, and cold antibodies were negative. Indirect antiglobulin test was positive, and antibody identification confirmed the presence of anti-C, anti-e, and anti-K. Her red cell phenotype was R2R2 and Kell negative (C-c+E+e-K-). The patient was started on erythropoietin. Requests for R2R2 and Kell negative units were sent to various blood banks across the country. After >800 A/O group units phenotyping and a waiting period of 6 weeks, two compatible R2R2 phenotypes and Kell negative could be arranged in-house and three units were received from Gurgaon, North India. Intraoperative management included blood preservation techniques including cell salvage, antifibrinolytic drug, and monitoring using thromboelastography. The estimated blood loss was 350 ml with pre- and postoperative Hb 10.4 gm% and 9.2 gm%, respectively. She received intraoperatively two units of single-donor platelet and four units of fresh frozen plasma and postoperatively one unit of leukocyte-depleted-packed red cells and doing well at 12-month follow-up. This case highlights the importance of advance immunohematology for timely detection of alloimmunization and providing antigen-negative compatible units, proper communication between the transfusion specialists, and the clinical team for proper patient blood management as well as the need for central rare donor registry program to avoid delays in providing compatible blood in such inevitable cases.

Ex situ liver resection and autotransplantation for advanced cholangiocarcinoma.

Advanced cholangiocarcinoma especially those involving the vasculature have extremely limited options of cure. Ex situ liver resection entails performing a total hepatectomy, resecting the tumour on the back-table followed by reimplantation (autotransplantation) of the liver. Application of this technique for these tumours has rarely been done due to complexity of the procedure and the dismal prognosis of the lesions. We present our experience of two cases of advanced intrahepatic cholangiocarcinoma with limited extrahepatic disease who underwent ex situ resection with autotransplantation. They underwent preoperative therapy with a waiting period to assess the tumour biology. Both patients underwent ex situ resection with extended hepatectomy on the back table. Both patients remain well on follow-up 24 months and 20 months, respectively, with excellent quality of life. Despite its technical complexity, ex situ liver resection may offer prolonged overall survival in selected patients with advanced cholangiocarcinoma and limited extrahepatic disease.

Paucity of Interlobular Bile Ducts in Multidrug-Resistant P-Glycoprotein 3 (MDR3) Deficiency.

Multidrug-resistant P-glycoprotein 3 (MDR3) is a phospholipid translocator encoded by the ABCB4 gene located on chromosome 7. MDR3 mediates the translocation of phosphatidylcholine across the canalicular membrane of the hepatocyte into bile. Severe MDR3 deficiency typically occurs during childhood with progressive cholestasis evolving to cirrhosis and liver failure, requiring liver transplantation. In this article, we report 2 pediatric cases of severe MDR3 deficiency with paucity of interlobular bile ducts. Both underwent living donor liver transplantation at our center for decompensated liver disease and portal hypertension. We diagnosed severe MDR3 deficiency in both the cases with negative MDR3 immunostaining in the explanted liver. Genetic studies revealed homozygous deletion single base pair deletion in exon 24 of the ABCB4 gene in the second child. The patients are on regular follow-up after liver transplant and are doing well. Our report highlights that cholangiopathy in MDR3 deficiency can lead to ductopenia in pediatric livers.

Liver transplantation for inferior vena cava leiomyosarcoma: from a Maslow's hammer to the Occam's razor.

Leiomyosarcoma (LMS) of primary vascular origin is a rare entity with only potentially curative option being complete surgical resection; despite which the prognosis remains dismal. Tumour recurrence is very common, and the benefits of adjuvant therapy are undefined. A 39-year-old woman presented with 6 months' history of abdominal pain, abdominal distension and pedal oedema. On evaluation, she was diagnosed to have chronic Budd-Chiari syndrome (BCS) secondary to a tumour arising from the inferior vena cava (IVC) on evaluation. Her liver decompensation included jaundice, gastrointestinal bleed and ascites. Following a detailed multidisciplinary team discussion, she underwent complete excision of the tumour along with a segment of the IVC with living donor liver transplantation. She remains disease-free 24 months following surgery. This is the first reported case of liver transplantation for IVC LMS causing chronic BCS.

Living donor liver transplantation and situs inversus totalis: cutting the Gordian knot.

Liver transplantation for biliary atresia splenic malformation syndrome associated with situs inversus totalis is a challenging task due to the complexity of associated malformations and the technical proficiency required to overcome them. We present the case of a 6-month-old infant who underwent liver transplantation for biliary atresia. A reduced left lateral segment liver graft from a live donor (his mother) was implanted. The postoperative period was uneventful, and the child remained well on follow-up. Thus, such rare congenital anomalies no longer prove to be a deterrent for successful liver transplantation.