A 27-YEAR EXPERIENCE WITH SPLENECTOMY FOR GAUCHERS-DISEASE
A 27-YEAR EXPERIENCE WITH SPLENECTOMY FOR GAUCHERS-DISEASE
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DOI：
10.1016/0002-9610(91)90363-i
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发表时间：
1991-01-01
影响因子：
3
通讯作者：
ELIAS, R
中科院分区：
文献类型：
--
作者：
FLESHNER, PR;AUFSES, AH;ELIAS, R
戈谢氏病是一种遗传性代谢性疾病，由酸性 β-葡萄糖碱活性缺陷以及由此导致的富含葡萄糖神经酰胺的巨噬细胞在肝脏、骨骼和脾脏中的积累引起。 脾切除术是戈谢病患者的首选治疗方法，这些患者出现脾肿大并伴有脾功能亢进和/或机械性压力症状。 我们对 1963 年 1 月至 1989 年 12 月期间在我们机构接受脾切除术的 48 名戈谢病患者的病历进行了分析，以确定该手术的短期和长期结果。 35 名 (73%) 患者接受了全脾切除术，而 13 名 (27%) 患者接受了部分脾切除术。 1 例术后死亡（全脾切除后），13 例患者（27%）出现术后并发症。 11 名患者 (23%) 在全脾切除术后出现加速性骨病（平均随访：96 个月）。 部分脾切除术的患者（平均随访：25 个月）没有出现进行性骨病。 手术后已有八名患者死亡。 所有四例因恶性疾病导致的死亡均发生在全脾切除术后的患者中。 迄今为止报道的最大规模的戈谢病脾切除术系列的结果证实，虽然可以以最小的发病率和死亡率进行全脾切除术或部分脾切除术，但全脾切除术伴随着更具侵袭性的骨病和恶性肿瘤的易感性。 需要前瞻性随机试验来证实部分脾切除术是否确实是戈谢病相关脾肿大的治疗选择。
Gaucher's disease is an inherited metabolic disorder caused by the defective activity of acid beta-glucosibase and the resultant accumulation of glucosyl ceramide-laden macrophages in the liver, bone, and spleen. Splenectomy is the preferred treatment for patients with Gaucher's disease who develop massive splenomegaly with accompanying hypersplenism and/or mechanical pressure symptoms. The charts of 48 patients with Gaucher's disease undergoing splenectomy at our institution between January 1963 and December 1989 were analyzed to determine the short- and long-term results of this procedure. Thirty-five (73%) patients had total splenectomy, whereas 13 (27%) patients had partial splenectomy. There was one postoperative death (after total splenectomy), and 13 patients (27%) had postoperative complications. Eleven patients (23%) presented with accelerated bone disease after total splenectomy (mean follow-up: 96 months). No patients having partial splenectomy (mean follow-up: 25 months) developed progressive bone disease. Eight patients have died since surgery. All four deaths due to malignant disease occurred in patients after total splenectomy. The results of this largest-ever reported series of splenectomy for Gaucher's disease confirm that while either total or partial splenectomy can be performed with minimal morbidity and mortality, total splenectomy is accompanied by more aggressive bone disease and a predisposition to malignancy. Prospective, randomized trials are needed to substantiate whether partial splenectomy is indeed the treatment of choice for splenomegaly associated with Gaucher's disease.