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...

Descripción completa

Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Future Science Ltd 2015
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5138004/
https://www.ncbi.nlm.nih.gov/pubmed/28031902
http://dx.doi.org/10.4155/fso.15.27
Descripción
Sumario: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.

Ejemplares similares