Aihara K, Azuma H, Ikeda Y, Akaike M, Abe M, Sugihara T, Matsumoto T
First Department of Internal Medicine, University of Tokushima School of Medicine, Japan.
Int J Hematol. 2001 Apr;73(3):351-5. doi: 10.1007/BF02981961.
Spur cell anemia, a hemolytic anemia observed in patients with alcoholic cirrhosis, is characterized by unusual erythrocyte morphology and an increased ratio of free cholesterol to phospholipid in the erythrocyte membrane. The prognosis of spur cell anemia is usually extremely poor, however, we describe here a patient with spur cell anemia who was successfully treated with combination therapy consisting of flunarizine, pentoxifylline, and cholestyramine. Initial therapy with flunarizine alone for 6 weeks did not significantly decrease the number of spur cells on peripheral blood smears. So pentoxifylline was added to the regimen. The patient recovered from the anemia, showed remarkable improvement with regard to the hyperbilirubinemia, and the changes were accompanied by a significant decrease in the number of spur cells in peripheral blood smears. To correct the hypercholesterolemia, cholestyramine was added to the regimen, which resulted in a reduction in the serum level of free cholesterol and an increase in the molar ratio of free cholesterol to phospholipid in erythrocyte membrane. However, 6 months later a skin eruption developed that was considered an adverse reaction to the drugs, so the flunarizine and pentoxifylline were discontinued. With cholestyramine therapy alone, the remission of spur cell anemia was maintained for more than 11 months. These observations suggest that non-invasive combination therapy with flunarizine, pentoxifylline, and cholestyramine is effective and valuable in the treatment of patients with spur cell anemia.
棘状细胞贫血是一种在酒精性肝硬化患者中观察到的溶血性贫血,其特征是红细胞形态异常以及红细胞膜中游离胆固醇与磷脂的比例增加。棘状细胞贫血的预后通常极差,然而,我们在此描述了一名棘状细胞贫血患者,他通过氟桂利嗪、己酮可可碱和考来烯胺联合治疗获得了成功。最初单独使用氟桂利嗪治疗6周并未显著减少外周血涂片上棘状细胞的数量。因此,将己酮可可碱添加到治疗方案中。患者贫血症状得到缓解,高胆红素血症有显著改善,同时外周血涂片上棘状细胞数量显著减少。为纠正高胆固醇血症,将考来烯胺添加到治疗方案中,这导致血清游离胆固醇水平降低,红细胞膜中游离胆固醇与磷脂的摩尔比增加。然而,6个月后出现了皮疹,被认为是药物不良反应,因此停用了氟桂利嗪和己酮可可碱。仅使用考来烯胺治疗,棘状细胞贫血的缓解维持了11个月以上。这些观察结果表明,氟桂利嗪、己酮可可碱和考来烯胺的非侵入性联合治疗在棘状细胞贫血患者的治疗中是有效且有价值的。