title: Secondary hemophagocytic syndrome: the importance of clinical suspicion.
abstract: Hemophagocytic syndrome is a rare and potentially fatal disorder characterized by pathological immune activation associated with a primary familial disorder, genetic mutations, or occurring as a sporadic condition. The latter can be secondary to infections, malignancies, or autoimmune diseases. Clinically, patients present signs of severe inflammation, with unremitting fever, cytopenias, spleen enlargement, phagocytosis of bone marrow elements, hypertriglyceridemia, and hypofibrinogenemia. Increased suspicion is determinant to timely initiate treatment in an attempt to alter the natural history. The authors present three clinical cases of this syndrome, with a brief review of the diagnostic criteria and treatment.
authors: Oncology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal.
authors: Cristina
authors: 0000-0002-5779-8020
authors: C
authors: Oliveira
authors: Hematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal.
authors: Sérgio
authors: 0000-0002-6316-8789
authors: S
authors: Chacim
authors: Hematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal.
authors: Isabel
authors: I
authors: Ferreira
authors: Hematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal.
authors: Nelson
authors: N
authors: Domingues
authors: Hematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal.
authors: José Mário
authors: JM
authors: Mariz