Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion

Michelle Likhtshteyn¹, Sadat Iqbal¹, Samy I. McFarlane^1, and Savanna Thor²

¹SUNY Downstate Medical Center, Brooklyn, NY, USA

²Department of Gastroenterology and Hepatology, SUNY Downstate Medical Center, Brooklyn, NY, USA

Cite this paper

Michelle Likhtshteyn, Sadat Iqbal, Samy I. McFarlane and Savanna Thor. Intrahepatic Cholestasis in a Sickle Cell Patient Unresponsive to Exchange Blood Transfusion. American Journal of Medical Case Reports. 2019; 7(4):67-70. doi: 10.12691/AJMCR-7-4-4

Abstract

With the advent of hydroxyurea, the sickle cell population has been enjoying a prolonged life span as compared to the pre-hydroxyurea era. Traditionally, acute complications of sickle cell disease includes acute chest syndrome, MI and stroke. In this report we present a case of an elderly man with sickle cell disease who presented with intrahepatic cholestasis (SCIC); a rather rare and fatal complication of sickle cell hemoglobinopathy. The patient presented with jaundice and elevated bilirubin up to 53, his hospital course was complicated by coagulopathy and encephalopathy, and expired on day 43 of presentation after failing multiple therapeutic interventions including exchange transfusion. In this report, we will provide literature review and discuss the underlying pathophysiologic mechanisms of intrahepatic cholestasis in the sickle cell population highlighting the need for immediate recognition and institution of therapy for this fatal complication of sickle cell disease, particularly in elderly populations with low metabolic reserve.

Keywords

liver transplantation, exchange transfusion, sickle cell, cholestasis, intrahepatic

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Lim M, Kimberly and Walter, MD, James W. Uncommon Hepatic Sequelae from an Acute Sickle Cell Crisis. The Medicine Forum. 2013; 14(17).
[2]	Hosiriluck N, Rassameehiran S, Argueta E, Tijani L. Reversal of liver function without exchange transfusion in sickle cell intrahepatic cholestasis. Proceedings (Baylor University Medical Center). 2014; 27(4): 361-363.
[3]	V. Papadopoulos AK, V. Papageorgiou, M. Topalidou, A. Mpellou, P. Patinakis, F. Girtovitis, D. Pantelidou and A. Kioumi. Successful Management of Sickle Cell Intrahepatic Cholestasis with Combined Use of Exchange Transfusion and Single-Pass Albumin Dialysis: A Case Report. Open Journal of Blood Diseases. 2013; 3(1): 36-42.
[4]	Brunetta DM, Silva-Pinto AC, Favarin de Macedo MdC, et al. Intrahepatic Cholestasis in Sickle Cell Disease: A Case Report. Anemia. 2011; 2011.
[5]	Papafragkakis H, Ona MA, Changela K, et al. Acute liver function decompensation in a patient with sickle cell disease managed with exchange transfusion and endoscopic retrograde cholangiography. Therapeutic Advances in Gastroenterology. 2014; 7(5): 217-223.
[6]	Banerjee S, Owen C, Chopra S. Sickle cell hepatopathy. Hepatology. 2001; 33(5): 1021-1028.
[7]	Khalifeh HK, Chamoun CT, Elhoujairy AH, Alkoussa WA, Lahoud CIZ, Masri GA. Acute Hepatic Crisis in Sickle Cell Anemia: Favorable Outcome After Exchange Transfusion. 2017.
[8]	Guimarães JAd, Silva LCdS. Sickle cell intrahepatic cholestasis unresponsive to exchange blood transfusion: a case report. Revista Brasileira de Hematologia e Hemoterapia. 2017.
[9]	Gardner K, Suddle A, Kane P, et al. How we treat sickle hepatopathy and liver transplantation in adults. Blood. 2014; 123(15): 2302-2307.
[10]	Costa, Daniel et al. “Case of Fatal Sickle Cell Intrahepatic Cholestasis Despite Use of Exchange Transfusion in an African-American.
[11]	Ahn H, Li CS, Wang W. Sickle cell hepatopathy: clinical presentation, treatment and outcome in pediatric and adult patients. Pediatric Blood Cancer. 2005; 45(2): 184-190.