JOURNAL ARTICLE

Secondary hemophagocytic syndrome: the importance of clinical suspicion

Cristina Oliveira, Sérgio Chacim, Isabel Ferreira, Nelson Domingues, José Mário Mariz
Case Reports in Hematology 2014, 2014: 958425
24963424
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.

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