Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report

Hemophagocytic lymphohystiocytosis is an extremely life-threatening immune deregulatory syndrome. It is characterized by pathologic activation of immune cells, leading to excessive cytokine production, multiorgan failure, and potentially, death. A 28-year-old primigravida at 32 weeks and 3 days of g...

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Autores principales: Bruce Lee, MD, Peter Kouides, MD, Stephanie Bousquet, MD, Paul Cabral, MD
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Lenguaje:EN
Publicado: Elsevier 2021
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spelling oai:doaj.org-article:4889ee099ed84e398de5cc65078f91442021-11-24T04:34:47ZEpstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report2666-577810.1016/j.xagr.2021.100024https://doaj.org/article/4889ee099ed84e398de5cc65078f91442021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2666577821000228https://doaj.org/toc/2666-5778Hemophagocytic lymphohystiocytosis is an extremely life-threatening immune deregulatory syndrome. It is characterized by pathologic activation of immune cells, leading to excessive cytokine production, multiorgan failure, and potentially, death. A 28-year-old primigravida at 32 weeks and 3 days of gestation presented with newly-diagnosed preeclampsia with severe features, fever, and fetal tachycardia. She was delivered by cesarean delivery. After delivery, she had a fever of unknown origin, with evidence of a hyperinflammatory state. Extensive infectious work-up was significant for positive Epstein-Barr Virus. A bone marrow biopsy demonstrated hemophagocytosis. She was diagnosed with Epstein-Barr-Virus-associated hemophagocytic lymphohystiocytosis and was treated with immunosuppression and chemotherapy. Hemophagocytic lymphohistiocytosis is a rare, life-threatening immune dysregulatory syndrome with both genetic and extragenic triggers that can occur in the postpartum period. Rituximab is an effective add-on therapy to conventional treatment.Bruce Lee, MDPeter Kouides, MDStephanie Bousquet, MDPaul Cabral, MDElsevierarticleEpstein-Barr virusfever of unknown originhemophagocytic lymphohistiocytosispostpartum preeclampsiaGynecology and obstetricsRG1-991ENAJOG Global Reports, Vol 1, Iss 4, Pp 100024- (2021)
institution DOAJ
collection DOAJ
language EN
topic Epstein-Barr virus
fever of unknown origin
hemophagocytic lymphohistiocytosis
postpartum preeclampsia
Gynecology and obstetrics
RG1-991
spellingShingle Epstein-Barr virus
fever of unknown origin
hemophagocytic lymphohistiocytosis
postpartum preeclampsia
Gynecology and obstetrics
RG1-991
Bruce Lee, MD
Peter Kouides, MD
Stephanie Bousquet, MD
Paul Cabral, MD
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
description Hemophagocytic lymphohystiocytosis is an extremely life-threatening immune deregulatory syndrome. It is characterized by pathologic activation of immune cells, leading to excessive cytokine production, multiorgan failure, and potentially, death. A 28-year-old primigravida at 32 weeks and 3 days of gestation presented with newly-diagnosed preeclampsia with severe features, fever, and fetal tachycardia. She was delivered by cesarean delivery. After delivery, she had a fever of unknown origin, with evidence of a hyperinflammatory state. Extensive infectious work-up was significant for positive Epstein-Barr Virus. A bone marrow biopsy demonstrated hemophagocytosis. She was diagnosed with Epstein-Barr-Virus-associated hemophagocytic lymphohystiocytosis and was treated with immunosuppression and chemotherapy. Hemophagocytic lymphohistiocytosis is a rare, life-threatening immune dysregulatory syndrome with both genetic and extragenic triggers that can occur in the postpartum period. Rituximab is an effective add-on therapy to conventional treatment.
format article
author Bruce Lee, MD
Peter Kouides, MD
Stephanie Bousquet, MD
Paul Cabral, MD
author_facet Bruce Lee, MD
Peter Kouides, MD
Stephanie Bousquet, MD
Paul Cabral, MD
author_sort Bruce Lee, MD
title Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
title_short Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
title_full Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
title_fullStr Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
title_full_unstemmed Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
title_sort epstein-barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report
publisher Elsevier
publishDate 2021
url https://doaj.org/article/4889ee099ed84e398de5cc65078f9144
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