Uemura K, Iinuma K, Ogata H, Taketomi T
Department of Lipid Biochemistry, Shinshu University School of Medicine, Matsumoto, Japan.
Jpn J Exp Med. 1987 Aug;57(4):223-30.
An unusual isohemagglutinin caused blood incompatibility in a patient with the Wiskott-Aldrich syndrome after nine transfusions. The patient's serum agglutinated erythrocytes of all the donors examined. The antibodies were cold agglutinins which agglutinated adult and cord erythrocytes equally. The binding of these antibodies to glycosphingolipids was analyzed by thin-layer chromatogram immunostaining and solid phase radioimmunoassay. The results indicate that the antibodies in the patient's serum bound specifically to lactonorhexaosylceramide and sialosyllactonorhexaosylceramide, glycolipids known to carry the i antigenic determinant. The antibodies did not bind to neolactotetraosylceramide, sialosylneolactotetraosylceramide or lactoisooctaosylceramide (I-active glycolipid). The patient's serum immunostained several components in the acidic glycolipids from OI erythrocytes. The antibody activity was predominantly present in IgG but the IgM showed similar binding specificity. Hemagglutination of OI erythrocytes with the patient's serum could be inhibited with lactonorhexaosylceramide. These results showed that the patient's antibodies recognized the i determinants carried by membrane surface glycolipids. The production of anti-i antibodies in the patient is intriguing in view of the decreased antibody response to carbohydrate antigens and deficient isohemagglutinins which have been generally observed in the Wiskott-Aldrich syndrome.
一种不寻常的同种血凝素在一名患有维斯科特-奥尔德里奇综合征的患者接受9次输血后导致了血液不相容。该患者的血清凝集了所有检测的供血者的红细胞。这些抗体是冷凝集素，能同等程度地凝集成人和脐带血红细胞。通过薄层色谱免疫染色和固相放射免疫测定分析了这些抗体与糖鞘脂的结合情况。结果表明，患者血清中的抗体特异性结合内酯神经六糖酰基神经酰胺和唾液酸内酯神经六糖酰基神经酰胺，这两种糖脂已知携带i抗原决定簇。这些抗体不与新乳糖四糖酰基神经酰胺、唾液酸新乳糖四糖酰基神经酰胺或乳糖异辛糖酰基神经酰胺（I活性糖脂）结合。患者血清对OI红细胞酸性糖脂中的几种成分进行了免疫染色。抗体活性主要存在于IgG中，但IgM显示出相似的结合特异性。患者血清对OI红细胞的血凝反应可被内酯神经六糖酰基神经酰胺抑制。这些结果表明，患者的抗体识别膜表面糖脂携带的i决定簇。鉴于维斯科特-奥尔德里奇综合征中普遍观察到的对碳水化合物抗原的抗体反应降低和同种血凝素缺乏，该患者产生抗-i抗体的情况很有趣。
