Spur-cell anemia in patient with acute-on-chronic liver failure: clinical case

One of the rare forms of anemia in patients with liver cirrhosis (LC) is acanthocytosis (spur cell anemia) – a non‑ immune hemolytic anemia caused by alterations in the lipid composition of the red blood cells membrane because of severe liver failure.

Case report. A 37‑year‑old patient with decompensated alcoholic LC (Child‑Pugh class C, the MELD‑Na (Model for End‑Stage Liver Disease – Na) score was 34 points) presented with severe weakness and dyspnea. Acute‑on‑chronic liver failure was diagnosed: CLIF‑C ACLF (Chronic Liver Failure Consortium Acute‑on‑Chronic Liver Failure Score) score was 46. Severe macrocytic anemia with reticulocytosis was detected: hemoglobin – 50 g/L, red blood cells – 1.26×10¹²/L, reticulocytes – 77.9 ‰. Other causes of anemia, such as blood loss, iron deficiency, vitamin B₁₂ and folate deficiencies were excluded. The Coombs test was negative, and bone marrow examination ruled out myelodysplasia. Blood smear analysis revealed that 20% of red blood cells had the shape of spur cells, with approximately half of them being acanthocytes. Orthotopic liver transplantation was performed. Follow-up examination after three months showed normalization of liver function tests and absence of anemia and acanthocytosis.

Discussion. This case report highlights the need for blood smear examination to detect acanthocytes – a rare but prognostically unfavorable cause of anemia in patients with LC. Liver transplantation remains the only effective treatment option.

1. Scheiner B., Semmler G., Maurer F., et al. Prevalence of and risk factors for anaemia in patients with advanced chronic liver disease. Liver Int. 2020 Jan; 40(1): 194–204. https://doi.org/10.1111/liv.14229. PMID: 31444993

2. Gonzalez-Casas R., Jones E.A., Moreno-Otero R. Spectrum of anemia associated with chronic liver disease. World J Gastroenterol. 2009 Oct; 15(37): 4653–4658. https://doi.org/10.3748/wjg.15.4653. PMID: 19787828

3. Sharma R., Holman C.J., Brown K.E. A thorny matter: Spur cell anemia. Ann Hepatol. 2023 Jan-Feb; 28(1): 100771. https://doi.org/10.1016/j.aohep.2022.100771. Epub 2022 Oct 12. PMID: 36241039

4. Roy A., Rodge G., Goenka M.K. Spur Cell Anaemia in Cirrhosis: A Narrative Review. J Clin Exp Hepatol. 2023 May-Jun; 13(3): 500–508. https://doi.org/10.1016/j.jceh.2022.10.005. Epub 2022 Oct 11. PMID: 37250881

5. Virk Z.M., Patel A.A., Leaf R.K., Al-Samkari H. Predictors of mortality and outcomes of liver transplant in spur cell hemolytic anemia. Am J Hematol. 2021 Dec; 96(12): 1611–1620. https://doi.org/10.1002/ajh.26359. Epub 2021 Oct 7. PMID: 34553418

6. Alexopoulou A., Vasilieva L., Kanellopoulou T., et al. Presence of spur cells as a highly predictive factor of mortality in patients with cirrhosis. J Gastroenterol Hepatol. 2014 Apr; 29(4): 830–834. https://doi.org/10.1111/jgh.12473. PMID: 24325340

7. Bevilacqua M., De Marco L., Stupia R., et al. Spur cells in liver cirrhosis are predictive of acute-on-chronic liver failure and liverrelated mortality regardless of severe anaemia. Intern Emerg Med. 2023 Aug; 18(5): 1397–1404. https://doi.org/10.1007/s11739-023-03303-x. Epub 2023 May 22. PMID: 37212944

8. Katiyar V., Dadlani A., Vohra I., et al. An Unusual Case of Hemolytic Anemia Reversed with Liver Transplantation. Int J Hematol Oncol Stem Cell Res. 2022 Apr; 16(2): 128–130. https://doi.org/10.18502/ijhoscr.v16i2.9206. PMID: 36304733

9. Fierro-Angulo O.M., González-Regueiro J.A., Pereira-García A., et al. Hematological abnormalities in liver cirrhosis. World J Hepatol. 2024 Sep; 16(9): 1229–1244. https://doi.org/10.4254/wjh.v16.i9.1229. PMID: 39351511

10. Perrin J., Georges A., Morali A., et al. Acanthocytes et hypocholestérolémie [Acanthocytes and hypocholesterolemia]. Ann Biol Clin (Paris). 2008 Sep-Oct; 66(5): 569–572. French. https://doi.org/10.1684/abc.2008.0269. PMID: 18957348

11. Foglia A. The acanthocyte-echinocyte differential: The example of chorea-acanthocytosis. Swiss Med Wkly. 2010 Jul; 140: 10.4414/ smw.2010.13039. https://doi.org/10.4414/smw.2010.13039. PMID: 20131113

12. Ribeiro R., Ferreira M., Coelho R., Pereira C. Zieve’s Syndrome: An Underdiagnosed Cause of Non-immune Hemolytic Anemia. Cureus. 2024 Jan; 16(1): e52034. https://doi.org/10.7759/cureus.52034. PMID: 38344483

13. Aihara K., Azuma H., Ikeda Y., et al. Successful combination therapy—flunarizine, pentoxifylline, and cholestyramine—for spur cell anemia. Int J Hematol. 2001 Apr; 73(3): 351–355. https://doi.org/10.1007/bf02981961. PMID: 11345202

14. Miki K., Maruki T., Imashuku S. Plasmapheresis for Spur Cell Anemia in a Patient with Alcoholic Liver Cirrhosis. Case Rep Hematol. 2018 Jun; 2018: 9513946. https://doi.org/10.1155/2018/9513946. PMID: 30034891

15. Piano S., Tonon M., Vettore E., et al. Incidence, predictors and outcomes of acute-on-chronic liver failure in outpatients with cirrhosis. J Hepatol. 2017 Dec; 67(6): 1177–1184. https://doi.org/10.1016