Secondary Hemophagocytic Syndrome in Lung Transplant Recipients: Report of 4 Cases

Purpose Secondary Haemophagocytic syndrome (HPS) is an uncommon clinic entity, with rapid evolution and potential cause of multiorgan failure and death. The most common cause are to herpes viridae infections, Epstein-Barr virus and cytomegalovirus Objective: Description of the clinical, laboratory data, cause and treatment characteristics of lung transplant recipients whit HPS in our unit. Methods We retrospectively analyzed 4 cases of HPS after lung transplantation (2 male, 2 female; age 45-68 years), during a 10 year period. The clinical and laboratory diagnostic criterial according to HLH-2004 guidelines were: fever, splenomegaly, cytopenia of 2 cell lines or more (hemoglobin, platelets, and neutrophils), hypertriglyceridemia and/or hypofibrinogenemia, elevated ferritin, elevated interleukin-2 receptor, decreased natural killer cell activity, and hemophagocytosis in bone marrow (>5/8 criteria). Results HPS developed 1 - 48 months in the posttransplant period. Three patients presented with fever and two whith hepatosplenomegaly. Laboratory tests showed pancytopenia, hypofibrinogenemia, elevated LDH, hyperferritinemia and hemophagocytosis in bone marrow and prolongation prothrombin time in all patients. Two patients had Epstein-Barr virus infection, 1 had Hepatitis B infection and 1 unknown cause. Three of the four patients died despite aggressive supportive therapy. Conclusion We report here the largest series of HPS after lung transplantation. In our experience the most common cause was Epstein-Barr virus infection.