Ibrahim EI Hassan1,2*, Christina Hajinicolaou1,3 and Priya Walabh1,2,4
1Department of Pediatrics and Child Health, School of Clinical Medicine, University of the Witwatersrand, Johannesburg, South Africa 2 Pediatric Gastroenterology, Hepatology and Nutrition Unit, Charlotte Maxeke Johannesburg Hospital, University of Witwatersrand, Johannesburg, South Africa 3 Divisional Head Pediatrics Gastroenterology, Chris Hani Baragwanath Hospital, University of Witwatersrand, Johannesburg, South Africa 4 Gauteng Provincial Solid Organ Transplant Division, South AfricaFulltext PDF
Introduction: Liver transplantation is considered a lifesaving procedure for patients with decompensated liver cirrhosis, when there are no available medical and surgical treatment options. The improvement in outcomes has led to the advent of an increased incidence of both acute and long-term complications after liver transplantation. Evans syndrome is a rare hematological disorder associated with a positive direct antiglobulin (Coombs) test, Autoimmune Hemolytic Anemia (AIHA) with Immune Thrombocytopenia (ITP) in the absence of any underlying etiology. Case Report: Here, we present a five-and-a-half-year-old patient who underwent a related livingdonor liver transplantation and presented with Evans syndrome as a post-transplant related complication. The patient was initially treated with prednisolone 2 mg/kg and during tapering had a relapse after which intravenous immunoglobulin was added to his therapy, with a good response. After a year of follow up, our patient maintained his hemoglobin level and platelet count on 0.25 mg/kg (5 mg) of prednisolone and tacrolimus as immunosuppressive medication. Our patient demonstrated a few transplant-related complications including Epstein Barr virus related post-transplant lymphoproliferative disorder and late portal vein thrombosis. These may have contributed to our patient developing Evans syndrome. Conclusion: There was an acceptable response to first-line therapy of Evans syndrome and close follow up is required to timeously identify relapses. Surgical options like splenectomy would be limited in our patient especially during the COVID-19 pandemic and shunt surgery for correction of the portal vein thrombosis is being considered.
Pediatric liver transplantation; Autoimmune hemolytic anemia; Portal vein thrombosis; Post-transplant lymphoproliferative disorder; COVID-19 pandemic; Splenectomy
EI Hassan I, Hajinicolaou C, Walabh P. Evans Syndrome: A Rare Complication of a Living Related Pediatric Liver Transplantation. Ann Digest Liver Dis. 2022; 4(1): 1019..