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纯合子β地中海贫血中的血清免疫球蛋白

Serum immunoglobulins in homozygous beta-thalassemia.

作者信息

Tovo P A, Miniero R, Barbera C, Sacchetti L, Saitta M

出版信息

Acta Haematol. 1981;65(1):21-5. doi: 10.1159/000207144.

DOI:10.1159/000207144
PMID:6791433
Abstract

IgG, IgA and IgM levels were studied in 187 homozygous beta-thalassemic patients and compared with age-matched normal control subjects. The not yet transfused and the polytransfused nonsplenectomized patients showed a significant increase of all Ig classes. The polytransfused splenectomized patients showed a significant increase only of IgG and IgA. The splenectomized patients, when compared to the nonsplenectomized ones, showed a significant increase of IgG, of IgA in the elder ones and a significant reduction of IgM. High ASLO and ASFLO titers were found especially in the splenectomized patients. The high Ig levels in younger not yet transfused patients, with little iron storage and normal hepatic enzyme values, demonstrate that transfusion therapy and liver damage do not play a main role in hypergammaglobulinemia. The absence of antimitochondrial, anticonnective tissue and homogeneous antinuclear antibodies seems to exclude a deficient suppressor mechanism. The hemocatheteric "overworking' of RES may reduce the antigen clearance with a consequent increase of antibody response. Splenectomy, by removing a consistent part of RES, enhances the phenomenon.

摘要

对187例纯合子β地中海贫血患者的IgG、IgA和IgM水平进行了研究,并与年龄匹配的正常对照者进行比较。未输血和多次输血且未行脾切除术的患者所有Ig类别均显著升高。多次输血并行脾切除术的患者仅IgG和IgA显著升高。与未行脾切除术的患者相比,行脾切除术的患者IgG显著升高,年长患者IgA显著升高,IgM显著降低。尤其在行脾切除术的患者中发现高抗链球菌溶血素O(ASLO)和抗链球菌溶血素O样物质(ASFLO)滴度。年龄较小、未输血、铁储存少且肝酶值正常的患者Ig水平高,表明输血治疗和肝损伤在高球蛋白血症中不起主要作用。不存在抗线粒体、抗结缔组织和均质抗核抗体似乎排除了抑制机制缺陷。网状内皮系统(RES)的血液导管“过度工作”可能会减少抗原清除,从而增加抗体反应。脾切除术通过切除RES的一部分,增强了这种现象。

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