Spur cell anemia related to alcoholic liver cirrhosis managed without liver transplantation: a case report and literature review

Abstract

Spur cell anemia is an acquired hemolytic anemia associated with liver cirrhosis and is characterized by the presence of increased large red blood cells, which are covered with spike-like projections that vary in width, length, and distribution. A 26-year-old man was referred to our hospital presenting with jaundice, lower limb edema, and dyspnea. The patient was subsequently diagnosed with spur cell anemia related to alcoholic liver cirrhosis. Spur cell anemia is an independent predictor of mortality in liver cirrhosis and has been associated with extremely poor prognosis. The most effective treatment for spur cell anemia is liver transplantation. As seen in the literature, the treatment of spur cell anemia without liver transplantation is quite challenging. This report highlights the importance of management and treatment strategies, including control of fluid retention, blood transfusion, plasma diafiltration, and administration of diuretics. Our treatment strategies might be useful in patients who are not candidate of liver transplantation or patients waiting for liver transplantation.

Keywords: Alcoholic liver cirrhosis; Case report; Liver cirrhosis; Plasma diafiltration; Spur cell anemia.

Fig. 1

Peripheral blood smear (May-Giemsa stain, × 1000) revealed approximately 25% of spur cells with multiple spicules irregularly distributed over the red blood cell

Fig. 2

a Chest computed tomography (CT) revealed pleural effusion and ground-glass opacity with partial consolidation suspecting pulmonary edema. b Contrast CT of the abdomen and pelvis revealed chronic liver disease and ascites estimated to be less than 1 L without portal vein thrombosis or hepatocellular carcinoma

Fig. 3

Pathological findings of liver biopsy. a (hematoxylin and eosin stain, scale bar: 50 μm): hematoxylin and eosin stain shows hepatocellular ballooning and Mallory bodies (blue arrowhead). b (periodic acid-Schiff stain, scale bar: 250 μm), c (Azan stain, scale: same as b): periodic acid-Schiff stain and Azan stain show lobular distortion with scattered small hepatic cell nests. Note that fatty change of hepatocyte is minimal

Fig. 4

Blood transfusion and fluid retention aggravated PaO₂/FiO₂ ratio. Intubation, plasma diafiltration (PDF) during blood transfusion, high-dose diuretics improved anemia, oxygenation, and body weight. Without liver transplantation, Model for End-stage Liver Disease (MELD) score and presence of spur cell in peripheral blood smear did not improve, although symptoms related to anemia and liver cirrhosis were successfully managed. FFP fresh frozen plasma, MELD Model for End-stage Liver Disease, PDF plasma diafiltration, RBC red blood cell, U Unit

Fig. 5

a Chest computed tomography (CT) at the time of discharge revealed improvement of pleural effusion and consolidation. b Abdominal CT at the time of discharge showed improved subcutaneous edema. Though, there was no obvious change in liver shape

Fig. 6

Blood transfusion is necessary for the management of spur cell anemia. Liver transplantation is the only curative treatment of spur cell anemia, considered by liver function, period of alcohol abstinence, and hepatocellular carcinoma. When the patient is not a candidate of liver transplantation, nutrition support for liver failure, respiratory care, including intubation, for respiratory failure, and fluid retention control, including diuretics, albumin infusion, plasma diafiltration, was helpful for management during blood transfusion