Freud E, Cohen I J, Mor C, Golinsky D, Blumenfeld A, Zer M
Department of Pediatric Surgery, Schneider Children's Medical Center of Israel, Petah Tiqva.
Blood Cells Mol Dis. 1998 Sep;24(3):309-16. doi: 10.1006/bcmd.1998.0198.
Partial splenectomy for Gaucher disease is often followed by reenlargement of the splenic remnant. It remains unclear if this process is due to tissue regeneration or to continued deposition of glucocerebroside in the reticuloendothelial system or both. We compared the splenic architecture before and after reenlargement in three cases of failed repeated partial splenectomy after two, six and five years. Using the number of lymphoid follicles per hundred low power fields (LF/LPF) as an arbitrary index, we found that prior to the first operation 18, 20 and 27 lymphoid follicles were present per one hundred low power fields, while at the second operation, the corresponding rates were 11, 15 and 17; in control spleens, an average of 712.5 lymphoid follicles were present in one hundred low power fields. The difference in the LF/LPF ratio before and after reenlargement, led us to speculate that splenic re-enlargement in Gaucher disease is mainly the result of the continued deposition of the glucocerebroside in the reticuloendothelial system of the splenic remnant, though some degree of true regeneration as well cannot be completely ruled out. These findings are compared with animal studies and results for partial splenectomy on humans, performed for trauma. Further studies in patients with Gaucher disease are warranted to better define the underlying mechanism of splenic reenlargement.
戈谢病患者行部分脾切除术后，脾残余常常会再次增大。目前尚不清楚这种过程是由于组织再生，还是由于葡萄糖脑苷脂在网状内皮系统中持续沉积，抑或是两者皆有。我们比较了3例分别在2年、6年和5年后重复部分脾切除失败的病例中，脾再次增大前后的脾结构。以每100个低倍视野中淋巴滤泡的数量（LF/LPF）作为一个任意指标，我们发现第一次手术前每100个低倍视野中有18个、20个和27个淋巴滤泡，而第二次手术时相应的比例分别为11个、15个和17个；在对照脾中，每100个低倍视野中平均有712.5个淋巴滤泡。再次增大前后LF/LPF比值的差异，使我们推测戈谢病中脾再次增大主要是由于葡萄糖脑苷脂在脾残余的网状内皮系统中持续沉积所致，不过也不能完全排除一定程度的真正再生。将这些发现与动物研究以及因创伤而行部分脾切除术的人类结果进行了比较。有必要对戈谢病患者进行进一步研究，以更好地明确脾再次增大的潜在机制。
