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August 1983

The Role of Splenectomy in Gaucher's Disease

Author Affiliations

From the Departments of Surgery B (Drs Shiloni, Bitran, and Durst) and Hematology (Dr Rachmilewitz), Hadassah University Hospital and Hebrew University—Hadassah Medical School, Jerusalem.

Arch Surg. 1983;118(8):929-932. 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 13kg. 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.

(Arch Surg 1983;118:929-932)