Acquired Hemophagocytic Syndrome in a Patient With Synovial Sarcoma: a Case Report

• DOI: 10.4155/fso.15.27
• Publication Date: 2015-06-26T14:16:19Z
• Authors: Chiara Ciccarese; Roberto Ferrara; Emanuela Fantinel; Camilla Zecchetto; Francesca Simionato; Elisabetta Grego; Silvia Ortolani; Mario Caccese; Davide Bimbatti; Sara Cingarlini; Matteo Brunelli; Angelo Andreini; Giampaolo Tortora; Francesco Massari
• Keywords: Hemophagocytosis; Hepatosplenomegaly; Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia low fibrinogen. Hemophagocytosis in the bone marrow hallmark of this syndrome. Based on pathogenetic mechanism, it can be classified into primary (inherited) or secondary (acquired) HLH. We report, our knowledge, first case acquired hemophagocytic that arose 20-year-old man affected synovial sarcoma as complication during chemotherapy.