Rare cause of non-immune hemolytic anemia in end-stage liver disease

Abstract

Spur cells are reportedly linked to advanced end-stage liver diseases and may lead to accelerated hemolysis. In this case report, we discuss one of these rare instances when a 45-year-old female with decompensated cirrhosis was admitted for severe anemia. Extensive workup revealed non-immune hemolysis secondary to spur cell formation. Orthotopic liver transplantation remains the only treatment of choice for reversal of spur cell anemia. Alternatively, multidrug therapy has also been explored, including usage of bile acid sequestrants; however, success is rare.

Keywords

Spur Cell Anemia / Acanthocytes / Orthotopic Liver Transplantation / SCA / Zieve Syndrome

References

[1] Douglass CC, McCall MS, Frenel EP. The acanthocyte in cirrhosis with hemolytic anemia. Ann Intern Med. 1968; 68(2): 390-7. PMid:5713921. https://doi.org/10.7326/0003-4819-68-2-390

[2] Keyserling K, Koprowski S. Spur-Cell Anemia. N Engl J Med. 2018; 379(8): 774. PMid:30134135. https://doi.org/10.1056/NEJMicm1714572

[3] Smith JA, Lonergan ET, Sterling K. Spur-Cell Anemia: Hemolytic Anemia with Red Cells Resembling Acanthocytes in Alcoholic Cirrhosis. N Engl J Med. 1964; 271: 396-8. PMid:14164657. https://doi.org/10.1056/NEJM196408202710804

[4] Murphy CH. Spur cell hemolytic anemia in liver failure. Transfusion. 2016; 56(4): 787-8. PMid:27079310. https://doi.org/10.1111/trf.13407

[5] Saibara T. Spur cells and acanthocytes in liver diseases. Hepatol Res. 2007; 37(6): 402-4. PMid:17539813. https://doi.org/10.1111/j.1872-034X.2007.00110.x

[6] Marks EI, Ollila TA. Acanthocytosis causing chronic hemolysis in a patient with advanced cirrhosis. Blood. 2019; 133(13): 1518. PMid:30923109. https://doi.org/10.1182/blood-2019-01-897892

[7] Marshall AT, Waxman I. Image of the month. Spur cell anemia. Gastroenterology. 1997; 112(2): 326-673. PMid:9024285. https://doi.org/10.1053/gast.1997.v112.agast970326

[8] Sugimoto S. Spur cell anemia associated with alcoholic cirrhosis. Intern Med. 2013; 52(24): 2831. PMid:24334595. https://doi.org/10.2169/internalmedicine.52.1335

[9] Malik P. Spur cell anemia in alcoholic cirrhosis: cure by orthotopic liver transplantation and recurrence after liver graft failure. Int Surg. 2002; 87(4): 201-4.

[10] Partain DK. Spur cell anemia in the setting of progressive liver allograft failure. Am J Hematol. 2016; 91(10): 1061. PMid:26874796. https://doi.org/10.1002/ajh.24331

[11] Liu MX. Hemolytic anemia in alcoholic liver disease: Zieve syndrome: A case report and literature review. Medicine (Baltimore). 2017; 96(47): e8742. PMid:29381966. https://doi.org/10.1097/MD.0000000000008742

[12] Shah R, Patel A, John S. Spur cell anemia in end-stage liver disease: a zebra! Am J Emerg Med. 2014; 32(8): 944 e3-4. PMid:24581882. https://doi.org/10.1016/j.ajem.2014.01.027