Mariette X
Service d'immuno-hématologie, hôpital Saint-Louis, Paris.
Rev Prat. 1993 Feb 1;43(3):281-4.
Monoclonal immunoglobulins present a normal structure and have an antibody activity. This activity may be directed against exo-antigens especially bacterial, but more frequently against auto-antigens. It may then be symptomatic and therefore explain unusual clinical or biological manifestations in the course of monoclonal dysglobulinemias. The most common auto-antibody activities are directed against red blood cells (responsible for cold agglutinin disease), IgG (leading to the formation of cryoglobulinemia), and myelin (responsible for peripheral neuropathy). Often, the monoclonal auto-antibody activity is asymptomatic and polyspecific, as it is only the monoclonal expression of polyclonal natural auto-antibodies. Genetic study of variable segments of heavy and light chains used by these monoclonal antibodies are useful to precise the role played by antigen in the driving of the lymphoid proliferation. These physiopathologic models concerning monoclonal gammapathies are a useful tool to understand the physiopathology of polyclonal auto-immune disease and the role of auto-antibodies.
单克隆免疫球蛋白具有正常结构并具有抗体活性。这种活性可能针对外源性抗原,尤其是细菌抗原,但更常见的是针对自身抗原。它可能会出现症状,从而解释单克隆球蛋白血症过程中不寻常的临床或生物学表现。最常见的自身抗体活性针对红细胞(导致冷凝集素病)、免疫球蛋白G(导致冷球蛋白血症的形成)和髓磷脂(导致周围神经病变)。通常,单克隆自身抗体活性无症状且具有多特异性,因为它只是多克隆天然自身抗体的单克隆表达。对这些单克隆抗体所使用的重链和轻链可变区进行基因研究,有助于明确抗原在驱动淋巴细胞增殖中所起的作用。这些关于单克隆丙种球蛋白病的病理生理模型是理解多克隆自身免疫性疾病的病理生理以及自身抗体作用的有用工具。