POLYCLONAL B-CELL LYMPHOCYTOSIS AND HYPERGAMMAGLOBULINEMIA IN PATIENTS WITH GAUCHER DISEASE
POLYCLONAL B-CELL LYMPHOCYTOSIS AND HYPERGAMMAGLOBULINEMIA IN PATIENTS WITH GAUCHER DISEASE
复制标题
DOI：
10.1002/ajh.2830290403
复制
发表时间：
1988-12-01
影响因子：
12.8
通讯作者：
GRALNICK, HR
中科院分区：
文献类型：
--
作者：
MARTI, GE;RYAN, ET;GRALNICK, HR
对23例戈谢病（GD）患者的血清进行了高丙种球蛋白血症、寡克隆和单克隆丙种球蛋白病分析。15/23例（65%）患者血清IgG水平升高，10/23例（43%）患者存在弥漫性高丙种球蛋白血症。寡克隆丙种球蛋白病在6例，单克隆丙种球蛋白病在2例。淋巴细胞亚群分析也进行了8个人与GD。5个人中有4个表现出增加的表面Ig阳性淋巴细胞，而7/7是积极的增加CD 19和/或CD 20阳性淋巴细胞。第八名患者被发现患有B细胞白血病。. kappa的统计分析和. da。直方图提示单克隆过量。然而，限制性内切酶分析的四个人与GD和增加B细胞未能显示任何证据的IG基因重排。血清IG异常和B细胞淋巴细胞增多似乎在Gd患者人群中很常见，与循环单克隆淋巴细胞无关。
Sera from 23 individuals with Gaucher disease (GD) were analyzed for hypergammaglobulinemia and oligoclonal and monoclonal gammopathies. Serum IgG level was elevated in 15/23 (65%) patients, and a diffuse hypergammaglobulinemia was present in 10/23 (43%) patients. An oligoclonal gammopathy was noted in six patients, and a monoclonal gammopathy in two. Lymphocyte subset analysis was also carried out in eight individuals with GD. Four of five individuals showed increased surface Ig-positive lymphocytes, while 7/7 were positive for either increased CD19- and/or CD20-positive lymphocytes. An eighth patient was found to have a B-cell leukemia. Statistical analysis of .kappa. and .lambda. histograms were suggestive of a monoclonal excess. However, restriction enzyme analysis of four individuals with GD and increased B cells failed to show any evidence of Ig gene rearrangements. Serum Ig abnormalities and perhaps B-cell lymphocytosis appear to be common in the Gd patient population and are not associated with circulating monoclonal lymphocytes.