Article, Hematology

Spur cell anemia in end-stage liver disease: a zebra!

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American Journal of Emergency Medicine

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Spur cell anemia in end-stage liver disease: a zebra!

Abstract

Anemia in alcoholic liver disease has a broad spectrum of differential diagnosis. One of the esoteric pathology that should be considered is spur cell anemia (SCA) in end-stage liver disease patients. Spur cell anemia is a rare type of hemolytic anemia with a grave prognosis. It closely resembles “Zieve syndrome,” which can present similarly and needs to be differentiated for prognostic and treatment purpose. Although presence of SCA indicates poor progno- sis in these patients, it is curable with liver transplant, and therefore, early diagnosis is crucial.

A 32-year-old female with a medical history of alcohol-induced liver cirrhosis, chronic Hepatitis C, malnutrition, and alcohol abuse presented with syncope. On admission, she complained of light- headedness, dizziness, and generalized fatigue. She also had nausea without vomiting. Our patient denied using alcohol and tobacco for 2 months and denied using any illicit drugs in the past 6 months. Family history was significant for colon cancer in both parents and no history of any liver disease. Her blood pressure on presentation was 88/58 mm Hg with a heart rate of 98 beats per minute. She was afebrile and not hypoxic. Pertinent physical examination findings were lethargy, alert to place and person, icterus, ascities with a fluid thrill, asterexis, and spider angiomas. Initial blood work showed normocytic anemia with hemoglobin level of 6.2, hematocrit of 17.4, white blood cells 9.7, and platelet count of 74,000. Her albumin was 2.7, alanine aminotransferase, aspartate aminotransferase, and alkaline phosphatase were 37, 58, and 118, respectively. Patient’s total bilirubin was 27.1 with direct bilirubin was 13.1. Her lactate dehydrogenase was 390, and haptoglobin was undetectable. Basic metabolic panel was remarkable of only hyponatremia of 125. Coagulation panel showed that prothrombin time, international normalized ratio, and partial thromboplastin time were 35.1, 3.37, and 57.9, respectively. Extensive anemia work up showed normal levels of iron, vitamin B12, and folate levels as well as negative autoimmune panel.

At this point, Zieve syndrome was considered a plausible diagnosis.

However, refuting our primary diagnosis, her Lipid panel showed total cholesterol of 97, triglycerides 61, low-density lipoprotein 34, very low- density lipoprotein 12, and high-density lipoprotein 51. Her peripheral blood smear showed spur cells with hemolyzed red blood cells (Fig.). After reviewing this, the diagnosis of “spur cell anemia (SCA) of end-stage liver disease (ESLD)” was given. She was then referred to the transplant center with periodic blood transfusion as needed in interim.

Anemia in alcoholic liver disease patients has wide variety of plausible etiologies. Possible causes range from malnutrition, iron, folate, or Vitamin B12 deficiency, bleeding, bone marrow suppression, and even splenic sequestration. Two rare pathologies for anemia in specifically advanced liver disease are Zieve syndrome and SCA. Zieve

syndrome is characterized by a triad of jaundice, dyslipidemia, and transient hemolytic anemia in alcoholic patients [1]. Spur cell anemia can mimic Zieve syndrome except for a few unique features. First, SCA is observed in very advance liver disease patients and can also be seen in nonalcoholic cirrhotic. Spur cell anemia lacks landmark features of Zieve syndrome such as fever and abdominal pain. In addition, patients have very low levels of total cholesterol as well as lipoproteins as oppose to high levels observed in Zieve syndrome. Finally, the spur cell appearance on a peripheral blood smear is absent in Zieve syndrome [2]. Appearance of SCA is considered a bad prognostic sign in patients with ESLD with the average survival as low as 12 days [3]. The only treatment options of SCA in ESLD patients remain orthotopic liver transplantation (OLT) so far [4]. Rapid resolution of SCA has been demonstrated after OLT in these patients in as soon as 6 days posttransplant [3].

It is imperative to identify the ominous signs for SCA in ESLD patients with anemia. Those who are candidate for transplant should be identified early, as this condition can be rapidly reversible after transplant.

Rushikesh Shah, MBBS

SUNY Upstate Medical University

Syracuse, NY 13202 E-mail address: [email protected]

Arpan Patel, MD Savio John, MBBS

SUNY Upstate Medical University

Syracuse, NY 13210

http://dx.doi.org/10.1016/j.ajem.2014.01.027

image of Fig

Fig. Spur cells on peripheral blood smear.

0735-6757/(C) 2014

References

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  2. Vassiliadis T, Mpoumponaris A, Vakalopoulou S, Giouleme O, Gkissakis D, Grammatikos N, et al. Spur cells and spur cell anemia in hospitalized patients with advanced liver disease: incidence and correlation with disease severity and survival. Hepatol Res

2010;40(2):161-70 http://dx.doi.org/10.1111/j.1872-034X.2009.00590.x. Epub 2010

Jan 11.

  1. Sousa JM, Giraldez A, De Blas JM, Diaz C, Pareja F, Serrano J, et al. Spur cell anemia in hepatic cirrhosis: incidence, prognosis and reversibility after liver transplantation. J Hepatol 2002;36:64.
  2. Aihara K, Azuma H, Ikeda Y, Akaike M, Abe M, Sugihara T, et al. Successful Combination therapy–flunarizine, pentoxifylline, and cholestyramine–for spur cell anemia. Int J Hematol 2001;73(3):351-5.