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Posttransplant Hemophagocytic Lymphohistiocytosis in Pediatric Liver Transplant Recipients

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Hemophagocytic lymphohistiocytosis (HLH) refers to the life-threatening syndrome of pathologic immune activation, with a characteristic constellation of clinical findings comprising prolonged fever, splenomegaly, cytopenias, elevated inflammatory markers, and hemophagocytosis (HP).(1)… Click to show full abstract

Hemophagocytic lymphohistiocytosis (HLH) refers to the life-threatening syndrome of pathologic immune activation, with a characteristic constellation of clinical findings comprising prolonged fever, splenomegaly, cytopenias, elevated inflammatory markers, and hemophagocytosis (HP).(1) It has been reported to occur after solid organ transplantation (SOT).(2) However, data are scarce regarding the incidence, characteristics, and long-term outcome of HLH in the post-liver transplantation (LT) setting, with only 5 pediatric and 18 adult cases reported in the literature.(3-7).

Keywords: liver; hemophagocytic lymphohistiocytosis; posttransplant hemophagocytic; lymphohistiocytosis pediatric; pediatric liver

Journal Title: Liver Transplantation
Year Published: 2020

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