Hardie R J
University Department of Clinical Neurology, National Hospital for Nervous Diseases, Queen Square, London.
Q J Med. 1989 Apr;71(264):291-306.
Acanthocytes have a distinct morphology and are not normally found in peripheral blood. They occur in association with at least three neurological syndromes. In abetalipoproteinaemia, a progressive spinocerebellar ataxia and retinopathy occurs secondary to malabsorption of vitamin E. Cases with chorea are often familial, with orofacial dyskinesia and an axonal neuropathy causing areflexia and muscle wasting. Areflexia and a subclinical myopathy also occur in the McLeod syndrome, in which there is abnormal expression of Kell blood group antigens. The exact mechanism of acanthocytosis in each disorder remains uncertain: passive changes in membrane lipids, surface receptor/ligand interactions, and a primary membrane defect are among the possibilities.
棘形红细胞具有独特的形态，正常情况下在外周血中不存在。它们与至少三种神经系统综合征相关。在无β脂蛋白血症中，由于维生素E吸收不良，会继发进行性脊髓小脑共济失调和视网膜病变。患有舞蹈症的病例通常具有家族性，伴有口面部运动障碍和轴索性神经病，导致反射消失和肌肉萎缩。反射消失和亚临床型肌病也见于麦克劳德综合征，该综合征中凯尔血型抗原表达异常。每种疾病中棘形红细胞增多的确切机制仍不确定：膜脂质的被动变化、表面受体/配体相互作用以及原发性膜缺陷都有可能。
