Treatment of hemophagocytic syndrome after living-related liver transplantation for fulminant liver failure in children

Abstract

Objective To summarize the clinical experience of successful treatment of hemophagncytic syndrome (HPS) arising after living-related liver transplantation (LRLT) for fulminant liver failure in children. Methods A 6 years old girl, who presented with serious jaundice, ascites and bloocling, was diagnosed as the fulminant liver failure of unknown etiology. LRLT was performed with left external lobe from his father in February 2008 in our hospital. Laboratory studies showed pancytopenia on day 3 after LRLT. On the 13th day, white blood cell count (WBC) and platelet (PLT) decreased to the lowest values, while the serum ferritin was elevated, epstein-barr virus-deoxyribonucleic acid (EBV-DNA) was positive by PCR and the bone marrow aspirate revealed many activated macrophages showing phagocytosis. The diagnosis of hemophagocytic syndrome was established. The patient was treated with cyclosporin A, dexamethasone, intravenous immunoglobulin (IVIg) and antiviral treatment with ganciclovir to suppress actively replicating EBV-infected cells. Results After LRLT, the patient's liver enzymes and the bilirubin (both direct and indirect) began to decrease on the 3rd day,approached to normal values on the 7th day,and returned to normal values completely within two weeks. The WBC and PLT began to increase on the 20th day, returned to normal values on the 40th day, serum ferritin and bone marrow aspirate returned to normal values completely. On the 40th day, the patient was permitted to discharge and follow-up. Up to now, the patient's condition is good. Conclusions HPS is a life-threatening hematological disorder and the outcome is usually fatal. When pancytopenia of unknown etiology with possible underlying infectious disease is observed in children with liver transplant recipients, HPS should be suspected and bone marrow cytological examination considered. Earlier diagnosis and effective treatment can gain good therapeutic results. Key words: Hemophagocytic syndromes; Liver transplantation; Hepatitis