Shiloni E, Bitran D, Rachmilewitz E, Durst A L
Arch Surg. 1983 Aug;118(8):929-32. doi: 10.1001/archsurg.1983.01390080037011.
Thirteen patients underwent splenectomy for Gaucher's disease. All patients were Jewish; 12, of Ashkenazi descent, had the chronic (type 1) form, and one child, of Sephardic (Persian) origin, probably had the intermediate (type 3) form. Hypersplenism was the indication for surgery in 11 patients, mechanical problems in the remaining two. The weight of the resected spleens ranged from 1.06 to 13 kg. Following surgery, hypersplenism (thrombocytopenia in particular) was improved, and the mechanical disturbances were relieved in all patients. There were no deaths and no morbidity related to the operative procedure. Long-term follow-up demonstrated progressive hepatomegaly without evidence of hepatic dysfunction in any of the patients. Bone marrow involvement manifested by osteoarticular complications appeared in five patients. Splenectomy is, we believe, a safe mode of treatment for type 1 Gaucher's disease.
13例患者因戈谢病接受了脾切除术。所有患者均为犹太人；12例为德系犹太人后裔，患慢性（1型）戈谢病，1例为西班牙系（波斯）裔儿童，可能患中间型（3型）戈谢病。脾功能亢进是11例患者的手术指征，其余2例是因机械性问题。切除脾脏的重量在1.06至13千克之间。术后，脾功能亢进（尤其是血小板减少症）得到改善，所有患者的机械性障碍均得到缓解。无手术相关死亡及发病率。长期随访显示，所有患者均出现进行性肝肿大，但无肝功能障碍证据。5例患者出现以骨关节并发症为表现的骨髓受累。我们认为，脾切除术是治疗1型戈谢病的一种安全治疗方式。
