Dijkhuis A M, Baggen M G, Montfoort A, Ouwendijk R J
Ikazia Ziekenhuis, afd. Inwendige Geneeskunde, Rotterdam.
Ned Tijdschr Geneeskd. 1991 Apr 20;135(16):716-8.
Spur-cell anaemia is a severe haemolytic disorder which may occur in patients with alcoholic liver cirrhosis. Characteristics are the bizarre shape of erythrocytes and the elevated cholesterol content in the membrane of the erythrocyte. The pathophysiology and possibilities for treatment are discussed with reference to a personal case. The patient described was treated with a cholesterol synthesis inhibitor, but no effect on the transfusion demand was noted. Splenectomy appears to be the best treatment. If splenectomy is not an option, frequent transfusions are the only therapy.
棘状细胞贫血是一种严重的溶血性疾病,可能发生在酒精性肝硬化患者身上。其特征是红细胞形状怪异以及红细胞膜中胆固醇含量升高。结合一个个案讨论了其病理生理学及治疗方法。所描述的患者接受了胆固醇合成抑制剂治疗,但未观察到对输血需求有任何影响。脾切除术似乎是最佳治疗方法。如果无法进行脾切除术,频繁输血是唯一的治疗手段。