Splenectomy as a diagnostic method in lymphoma-associated hemophagocytic lymphohistiocytosis of unknown origin

摘要

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and life-threatening disease caused by a highly active but ineffective immune response. 1 Uncontrolled immune activation results in the typical clinical characteristics and laboratory findings of HLH, including high fever, pancytopenia, hepatosplenomegaly, liver dysfunction, coagulopathy, hyperferritinaemia and hemophagocytosis in the bone marrow or other organs. HLH may be divided into primary (familial) and secondary subtypes.