Cargando…

Multiorgan failure due to hemophagocytic syndrome: A case report

INTRODUCTION: Hemophagocytic syndrome (HFS) is a potentially lethal disorder due to an uncontrolled immune response to a triggering agent. Our objective is to raise the importance of HFS early diagnosis by presenting a representative case. CASE PRESENTATION: A sixteen-year-old girl with Still diseas...

Descripción completa

Detalles Bibliográficos
Autores principales: Mayordomo-Colunga, Juan, Rey, Corsino, González, Soledad, Concha, Andrés
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2566570/
https://www.ncbi.nlm.nih.gov/pubmed/18834507
http://dx.doi.org/10.1186/1757-1626-1-209
_version_ 1782159950019035136
author Mayordomo-Colunga, Juan
Rey, Corsino
González, Soledad
Concha, Andrés
author_facet Mayordomo-Colunga, Juan
Rey, Corsino
González, Soledad
Concha, Andrés
author_sort Mayordomo-Colunga, Juan
collection PubMed
description INTRODUCTION: Hemophagocytic syndrome (HFS) is a potentially lethal disorder due to an uncontrolled immune response to a triggering agent. Our objective is to raise the importance of HFS early diagnosis by presenting a representative case. CASE PRESENTATION: A sixteen-year-old girl with Still disease diagnosis developed a progressive multiorgan failure including acute respiratory distress (ARDS), anemia and thrombopenia, elevated liver enzymes, renal failure, coagulopathy with hypofibrinogenemia, and acute phase reactants elevation despite broad-spectrum antibiotics. A bone marrow puncture-biopsy was performed, and hemophagocytosis was found. Prolonged fever, splenomegaly, bicytopenia, hypofibrinogenemia, hyperferritinemia and hypertriglyceridemia confirmed HFS diagnosis. She received intensive care support therapy including mechanical ventilation and specific therapy according to HLH 2004 protocol, with a very good response. CONCLUSION: Our case shows complexity of HFS diagnosis, due to septic shock-like manifestations. Early diagnosis is essential to start appropriate treatment achieving a better outcome.
format Text
id pubmed-2566570
institution National Center for Biotechnology Information
language English
publishDate 2008
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-25665702008-10-11 Multiorgan failure due to hemophagocytic syndrome: A case report Mayordomo-Colunga, Juan Rey, Corsino González, Soledad Concha, Andrés Cases J Case Report INTRODUCTION: Hemophagocytic syndrome (HFS) is a potentially lethal disorder due to an uncontrolled immune response to a triggering agent. Our objective is to raise the importance of HFS early diagnosis by presenting a representative case. CASE PRESENTATION: A sixteen-year-old girl with Still disease diagnosis developed a progressive multiorgan failure including acute respiratory distress (ARDS), anemia and thrombopenia, elevated liver enzymes, renal failure, coagulopathy with hypofibrinogenemia, and acute phase reactants elevation despite broad-spectrum antibiotics. A bone marrow puncture-biopsy was performed, and hemophagocytosis was found. Prolonged fever, splenomegaly, bicytopenia, hypofibrinogenemia, hyperferritinemia and hypertriglyceridemia confirmed HFS diagnosis. She received intensive care support therapy including mechanical ventilation and specific therapy according to HLH 2004 protocol, with a very good response. CONCLUSION: Our case shows complexity of HFS diagnosis, due to septic shock-like manifestations. Early diagnosis is essential to start appropriate treatment achieving a better outcome. BioMed Central 2008-10-03 /pmc/articles/PMC2566570/ /pubmed/18834507 http://dx.doi.org/10.1186/1757-1626-1-209 Text en Copyright © 2008 Mayordomo-Colunga et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Mayordomo-Colunga, Juan
Rey, Corsino
González, Soledad
Concha, Andrés
Multiorgan failure due to hemophagocytic syndrome: A case report
title Multiorgan failure due to hemophagocytic syndrome: A case report
title_full Multiorgan failure due to hemophagocytic syndrome: A case report
title_fullStr Multiorgan failure due to hemophagocytic syndrome: A case report
title_full_unstemmed Multiorgan failure due to hemophagocytic syndrome: A case report
title_short Multiorgan failure due to hemophagocytic syndrome: A case report
title_sort multiorgan failure due to hemophagocytic syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2566570/
https://www.ncbi.nlm.nih.gov/pubmed/18834507
http://dx.doi.org/10.1186/1757-1626-1-209
work_keys_str_mv AT mayordomocolungajuan multiorganfailureduetohemophagocyticsyndromeacasereport
AT reycorsino multiorganfailureduetohemophagocyticsyndromeacasereport
AT gonzalezsoledad multiorganfailureduetohemophagocyticsyndromeacasereport
AT conchaandres multiorganfailureduetohemophagocyticsyndromeacasereport