Raffa Gabriella A, Byrnes Diana M, Byrnes John J
Internal Medicine Residency Program, Jackson Memorial Hospital, Miami, FL, USA.
Hematology/Oncology Fellowship Program, Jackson Memorial Hospital, Miami, FL, USA.
Case Rep Hematol. 2021 Mar 26;2021:8883335. doi: 10.1155/2021/8883335. eCollection 2021.
The etiology of anemia in liver cirrhosis is multifactorial; one less recognized cause is hemolytic anemia due to spur cells, known as spur cell anemia. We present the case of a 57-year-old woman with alcoholic cirrhosis who presented with symptomatic macrocytic anemia with a hemoglobin level of 7.4 g/dL and signs of decompensated liver disease. Notably, she had no signs of overt bleeding. Further workup was consistent with hemolysis, with peripheral smear demonstrating spur cells. The patient was treated with both steroids and IVIG, although she eventually expired. The characteristic morphology of spur cells is due to alteration of the lipid composition of the erythrocyte membrane, changing its shape and leading to splenic sequestration and destruction. Characteristic of this disorder is an increased ratio of cholesterol to phospholipid on the membrane, as well as low levels of apolipoproteins and low- and high-density lipoproteins. The presence of spur cells is an indicator of poor prognosis and high risk of mortality. Currently, the only definitive cure is liver transplantation. There is a paucity of literature on the prevalence of this phenomenon and even less about treatment. This case highlights the importance of recognition of spur cell anemia as a cause of anemia in cirrhosis as well as the importance of the peripheral smear in the diagnostic workup. Early recognition can lead to avoidance of unnecessary procedures. Further research is needed to elucidate the true prevalence of spur cell anemia and examine further treatment options.
肝硬化贫血的病因是多因素的;一个较少被认识到的原因是由于棘形细胞导致的溶血性贫血,即棘形细胞贫血。我们报告一例57岁酒精性肝硬化女性病例,该患者出现有症状的大细胞性贫血,血红蛋白水平为7.4g/dL,并有失代偿性肝病的体征。值得注意的是,她没有明显出血的迹象。进一步检查与溶血相符,外周血涂片显示有棘形细胞。患者接受了类固醇和静脉注射免疫球蛋白治疗,尽管她最终还是去世了。棘形细胞的特征形态是由于红细胞膜脂质成分的改变,改变了其形状,导致脾脏扣押和破坏。这种疾病的特征是膜上胆固醇与磷脂的比例增加,以及载脂蛋白和低密度及高密度脂蛋白水平降低。棘形细胞的存在是预后不良和高死亡风险的一个指标。目前,唯一确切的治愈方法是肝移植。关于这种现象的患病率的文献很少,关于治疗的更少。该病例强调了认识到棘形细胞贫血是肝硬化贫血原因的重要性,以及外周血涂片在诊断检查中的重要性。早期识别可以避免不必要的检查。需要进一步研究以阐明棘形细胞贫血的真实患病率并研究更多的治疗选择。