Immunodeficiency in alpha-mannosidosis:: a matched case-control study on immunoglobulins, complement factors, receptor density, phagocytosis and intracellular killing in leucocytes
Immunodeficiency in alpha-mannosidosis:: a matched case-control study on immunoglobulins, complement factors, receptor density, phagocytosis and intracellular killing in leucocytes
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DOI：
10.1007/s004310000545
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发表时间：
2000-09-01
影响因子：
3.6
通讯作者：
Sjursen, H
中科院分区：
文献类型：
--
作者：
Malm, D;Halvorsen, DS;Sjursen, H
常染色体隐性溶酶体贮积病-甘露甘露病患者有反复感染。为了研究这种免疫缺陷的机制，6名患者与6名健康对照者进行了体液和细胞免疫能力的研究。包括b细胞在内的循环白细胞数量没有差异？观察免疫球蛋白主类水平，不观察IgG亚类水平。然而，免疫后脊髓灰质炎病毒特异性抗体的血清水平。白喉毒素和破伤风毒素明显减少。在患者中，补体结合受体CD11b和fc受体CD16在单核细胞和多形核中性粒细胞（PMN）上的密度显著增强，在混合人血清存在下，吞噬PMN的数量显著增加。在自体血清中没有观察到这一点，表明听觉特性发生了改变。在正常的PMN中，吞噬作用也被患者血清中的一种因子所抑制。尽管维持氧化破裂，患者PMN显示细胞内细菌杀灭不足。结论我们的数据表明，α -甘露甘露病患者在体液和细胞水平上都存在免疫缺陷。
Patients with the autosomal recessive lysosomal storage disease alpha-mannosidosis suffer from recurrent infections. To study the mechanisms of this immunodeficiency, six patients were matched against six healthy controls and their humoral and cellular immunocompetence investigated. No differences in the number of circulating leucocytes including B-cells? levels of immunoglobulin main classes, nor IgG subclasses were observed. However, post-immunisation serum levels of specific antibodies against poliovirus. diphtheria toxin and tetanus toxin were significantly reduced. In patients, the density of the complement-binding receptor CD11b and the Fc-receptor CD16 was significantly enhanced on monocytes and polymorphonuclear neutrophils (PMN) and the number of phagocytosing PMN was significantly increased in the presence of pooled human serum. This was not observed in the presence of autologous serum, indicating altered opsonic properties. Also in normal PMN, phagocytosis was inhibited by a factor in the serum from the patients. Despite maintained oxidative burst, patient PMN demonstrated insufficient intracellular bacterial killing.Conclusion Our data indicate that patients with alpha-mannosidosis have an immunodeficiency at both the humoral and cellular level.