Friedman E W, Williams J C, Van Hook L
Department of Medicine, Montefiore-North Central Bronx Hospital, New York 10467.
Am J Med. 1988 Mar;84(3 Pt 1):513-6. doi: 10.1016/0002-9343(88)90275-6.
Five patients with hereditary spherocytosis diagnosed in their seventh to ninth decades of life are presented. These patients are remarkable for absent or mild clinical manifestations of disease. Splenectomy is the recommended treatment for hereditary spherocytosis to avoid the complications of aplastic or hemolytic crisis. When the diagnosis is made in the elderly, the treatment of choice may be careful observation with folic acid supplementation rather than splenectomy. This recommendation is based on the incidence of complications of splenectomy in the elderly in comparison to the severity and incidence of complications from the disease itself.
本文介绍了5例在70至90岁被诊断为遗传性球形红细胞增多症的患者。这些患者疾病的临床表现缺失或轻微,十分引人注目。脾切除术是遗传性球形红细胞增多症的推荐治疗方法,以避免再生障碍性或溶血性危机的并发症。当在老年人中做出诊断时,治疗的选择可能是谨慎观察并补充叶酸,而不是脾切除术。这一建议是基于老年人脾切除术后并发症的发生率与疾病本身并发症的严重程度和发生率相比较得出的。