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Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion

Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe h...

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Autores principales: Adkins, Brian D., Savani, Bipin N., Booth, Garrett S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Atlantis Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432363/
https://www.ncbi.nlm.nih.gov/pubmed/34595422
http://dx.doi.org/10.2991/chi.d.190630.001
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author Adkins, Brian D.
Savani, Bipin N.
Booth, Garrett S.
author_facet Adkins, Brian D.
Savani, Bipin N.
Booth, Garrett S.
author_sort Adkins, Brian D.
collection PubMed
description Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe hepatic crisis wherein sinusoidal red cell sickling leads to massive hepatocyte dysfunction and cholestatic laboratory findings. Acute SCIC is defined by abdominal pain with progressive hepatic injury associated with hyperbilirubinemia, renal failure, encephalopathy, and coagulopathy. Patients are generally managed with red blood cell exchange transfusion (RBCEx), when available, as this is a potentially fatal condition. Simple transfusion may be utilized in resource-poor environment or when patients refuse RBCEx. As less than 50 adult cases have been described in the literature, many of them with limited follow-up, randomized clinical trials comparing RBCEx with other treatments are currently unfeasible. Likewise, a chronic form exists, but is less well characterized, and is associated with persistent bilirubinemia and a variable course in terms of progressive hepatic disease. We undertake a brief review of the literature and discuss two cases of SCIC managed with RBCEx at our institution.
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spelling pubmed-84323632021-09-29 Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion Adkins, Brian D. Savani, Bipin N. Booth, Garrett S. Clin Hematol Int Perspective Sickle cell disease patients are commonly treated at transfusion medicine services, and understanding of the hepatic manifestations of the disease is key for optimal management, specifically, in individuals presenting with sickle cell intrahepatic cholestasis (SCIC). SCIC represents a rare, severe hepatic crisis wherein sinusoidal red cell sickling leads to massive hepatocyte dysfunction and cholestatic laboratory findings. Acute SCIC is defined by abdominal pain with progressive hepatic injury associated with hyperbilirubinemia, renal failure, encephalopathy, and coagulopathy. Patients are generally managed with red blood cell exchange transfusion (RBCEx), when available, as this is a potentially fatal condition. Simple transfusion may be utilized in resource-poor environment or when patients refuse RBCEx. As less than 50 adult cases have been described in the literature, many of them with limited follow-up, randomized clinical trials comparing RBCEx with other treatments are currently unfeasible. Likewise, a chronic form exists, but is less well characterized, and is associated with persistent bilirubinemia and a variable course in terms of progressive hepatic disease. We undertake a brief review of the literature and discuss two cases of SCIC managed with RBCEx at our institution. Atlantis Press 2019-09-01 /pmc/articles/PMC8432363/ /pubmed/34595422 http://dx.doi.org/10.2991/chi.d.190630.001 Text en © 2019 International Academy for Clinical Hematology. Publishing services by Atlantis Press International B.V. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed under the CC BY-NC 4.0 license (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ).
spellingShingle Perspective
Adkins, Brian D.
Savani, Bipin N.
Booth, Garrett S.
Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion
title Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion
title_full Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion
title_fullStr Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion
title_full_unstemmed Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion
title_short Management of Sickle Cell Intrahepatic Cholestasis: An Argument in Favor of Automated Exchange Transfusion
title_sort management of sickle cell intrahepatic cholestasis: an argument in favor of automated exchange transfusion
topic Perspective
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432363/
https://www.ncbi.nlm.nih.gov/pubmed/34595422
http://dx.doi.org/10.2991/chi.d.190630.001
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