Acquired hemophagocytic syndrome in a patient with synovial sarcoma: a case report

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglycer...

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Published in:Future science OA 2015-11, Vol.1 (4), p.FSO29
Main Authors: Ciccarese, Chiara, Ferrara, Roberto, Fantinel, Emanuela, Zecchetto, Camilla, Simionato, Francesca, Grego, Elisabetta, Ortolani, Silvia, Caccese, Mario, Bimbatti, Davide, Cingarlini, Sara, Brunelli, Matteo, Andreini, Angelo, Tortora, Giampaolo, Massari, Francesco
Format: Article
Language:English
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Case Report
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Summary:Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by severe hyperinflammation due to an overwhelming ineffective immune response to different triggers. Most important symptoms are fever, hepatosplenomegaly and cytopenias. Biochemical signs include elevated ferritin, hypertriglyceridemia and low fibrinogen. Hemophagocytosis in the bone marrow is a hallmark of this syndrome. Based on the pathogenetic mechanism, it can be classified into primary (inherited) or secondary (acquired) HLH. We report, to our knowledge, the first case of acquired hemophagocytic syndrome that arose in a 20-year-old man affected by synovial sarcoma as a complication during chemotherapy.
ISSN:2056-5623
2056-5623
DOI:10.4155/fso.15.27