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Case Reports in Hematology
Volume 2014 (2014), Article ID 958425, 5 pages
http://dx.doi.org/10.1155/2014/958425
Case Report

Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion

1Oncology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal
2Hematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, Portugal

Received 3 March 2014; Revised 22 April 2014; Accepted 6 May 2014; Published 19 May 2014

Academic Editor: Panagiotis Tsirigotis

Copyright © 2014 Cristina Oliveira et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.