Case of fatal sickle cell intrahepatic cholestasis despite use of exchange transfusion in an African-American patient

Journal of the National Medical Association

Volumn 98, Issue 7, 2006, Pages 1183-1187

  Costa, Daniel B ^a,b   Miksad, Rebecca A ^a,b   Buff, Michael S ^a,b   Wang, Yihong ^a,b   Dezube, Bruce J ^a,b,c  

^a Harvard Medical School ^*

^b HARVARD MEDICAL SCHOOL   (United States)

^c BETH ISRAEL DEACONESS MEDICAL CENTER   (United States)

Author keywords

Cirrhosis; Exchange transfusion; Hepatitis C; Sickle cell disease; Sickle cell intrahepatic cholestasis

Indexed keywords

BILIRUBIN; BILIRUBIN GLUCURONIDE; HEMOGLOBIN S;

ADULT; AFRICAN AMERICAN; ARTICLE; AUTOPSY; BETA THALASSEMIA; BILIRUBIN BLOOD LEVEL; CASE REPORT; COMMON BILE DUCT STONE; ERYTHROCYTE TRANSFUSION; EXCHANGE BLOOD TRANSFUSION; HEPATITIS C; HUMAN; HYPERBILIRUBINEMIA; INTRAHEPATIC CHOLESTASIS; KIDNEY FAILURE; LIVER CIRRHOSIS; LIVER FAILURE; MALE; PRIORITY JOURNAL; SICKLE CELL ANEMIA; SICKLE CELL INTRAHEPATIC CHOLESTASIS;

AFRICAN AMERICANS; ANEMIA, SICKLE CELL; BETA-THALASSEMIA; CHOLESTASIS, INTRAHEPATIC; COMORBIDITY; ERYTHROCYTE TRANSFUSION; EXCHANGE TRANSFUSION, WHOLE BLOOD; FATAL OUTCOME; FIBROSIS; HEPATITIS C; HUMANS; KIDNEY FAILURE; LIVER; LIVER FAILURE; MALE; MIDDLE AGED;

EID: 33746774711     PISSN: 00279684     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (16)

1. Banerjee S, Owen C, Chopra S. Sickle cell hepatopathy. Hepatology. 2001;33:1022-1028.
2. Sheehy TW. Sickle cell hepatopathy. South Med J. 1977;70:533-538.
3. Sheehy TW, Law DE, Wade BH. Exchange transfusion for sickle cell intrahepatic cholestasis. Arch Intern Med. 1980;140:1364-1366.
4. Shao SH, Orringer EP. Sickle cell intrahepatic cholestasis: approach to a difficult problem. Am J Gastroenterol. 1995;90:2048-2050.
5. Gilli SC, Boin IF, Sergio Leonardi L, et al. Liver transplantation in a patient with S(beta)0-thalassemia. Transplantation. 2002;74:896-898.
6. Khurshid I, Anderson L, Downie GH, et al. Sickle cell disease, extreme hyperbilirubinemia, and pericardial tamponade: case report and review of literature. Crit Care Med. 2002;30:2363-2367.
7. Klion FM, Weigner MJ, Schaffner F. Cholestasis in sickle cell anemia. Am J Med. 1964;37:829-832.
8. Owen DM, Aldridge JE, Thompson RB. An unusual hepatic sequela of sickle cell anemia: a report of five cases. Am J Med Sci. 1965;249:175-185.
9. Bauer TW, Moore GW, Hutchins GM. The liver in sickle cell disease: a clinicopathologic study of 70 patients. Am J Med. 1980;69:833-837.
10. Betrosian A, Balla M, Kafiri G, et al. Reversal of liver failure in sickle cell vaso-occlusive crisis. Am J Med Sci. 1996;311:292-295.
11. Stephan JL, Merpit-Gonon E, Richard O, et al. Fulminant liver failure in a 12-year-old girl with sickle cell anemia: favorable outcome after exchange transfusion. Eur J Pediatr. 1995;154:469-471.
12. Svarch E, Gonzalez A, Villaescusa R, et al. Plasma exchange for acute cholestasis in homozygous sickle cell disease. Haematologia. 1986;19:49-51.
13. Morrow JD, McKenzie SW. Survival after intrahepatic cholestasis associated with sickle cell disease. J Tenn Med Assoc. 1986;79:199-200.
14. O'Callaghan A, O'Brien SG, Nikovic M, et al. Chronic intrahepatic cholestasis in sickle cell disease requiring exchange transfusion. Gut. 1995;37:144-147.
15. Emre S, Schwartz ME, Schneider B, et al. Living related transplantation for acute liver failure in children. Liver Transpl Surg. 1999;5:161-165.
16. Zakaria N, Knisely A, Portmann B, et al. Acute sickle cell hepatopathy represents a potential contraindication for percutaneous liver biopsy. Blood. 2003;101:101-103.