Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion

Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion

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. Clin...

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Main Authors: Cristina Oliveira, Sérgio Chacim, Isabel Ferreira, Nelson Domingues, José Mário Mariz
Format: Article
Language:English
Published: Hindawi Limited 2014-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2014/958425
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spelling doaj-6b38da2650f74004bbcaa4a9a19a96ee2020-11-24T23:25:47ZengHindawi LimitedCase Reports in Hematology2090-65602090-65792014-01-01201410.1155/2014/958425958425Secondary Hemophagocytic Syndrome: The Importance of Clinical SuspicionCristina Oliveira0Sérgio Chacim1Isabel Ferreira2Nelson Domingues3José Mário Mariz4Oncology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, PortugalHematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, PortugalHematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, PortugalHematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, PortugalHematology Department, Portuguese Institute of Oncology of Porto, Rua Dr. António Bernardino de Almeida, 4200-072 Porto, PortugalHemophagocytic 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.http://dx.doi.org/10.1155/2014/958425
collection DOAJ
language English
format Article
sources DOAJ
author Cristina Oliveira
Sérgio Chacim
Isabel Ferreira
Nelson Domingues
José Mário Mariz
spellingShingle Cristina Oliveira
Sérgio Chacim
Isabel Ferreira
Nelson Domingues
José Mário Mariz
Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion
Case Reports in Hematology
author_facet Cristina Oliveira
Sérgio Chacim
Isabel Ferreira
Nelson Domingues
José Mário Mariz
author_sort Cristina Oliveira
title Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion
title_short Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion
title_full Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion
title_fullStr Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion
title_full_unstemmed Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion
title_sort secondary hemophagocytic syndrome: the importance of clinical suspicion
publisher Hindawi Limited
series Case Reports in Hematology
issn 2090-6560
2090-6579
publishDate 2014-01-01
description 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.
url http://dx.doi.org/10.1155/2014/958425
work_keys_str_mv AT cristinaoliveira secondaryhemophagocyticsyndrometheimportanceofclinicalsuspicion
AT sergiochacim secondaryhemophagocyticsyndrometheimportanceofclinicalsuspicion
AT isabelferreira secondaryhemophagocyticsyndrometheimportanceofclinicalsuspicion
AT nelsondomingues secondaryhemophagocyticsyndrometheimportanceofclinicalsuspicion
AT josemariomariz secondaryhemophagocyticsyndrometheimportanceofclinicalsuspicion
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