Steffen D J, Elliott G S, Leipold H W, Smith J E
Department of Pathology, College of Veterinary Medicine, Kansas State University, Manhattan 66506.
J Vet Diagn Invest. 1992 Jan;4(1):31-7. doi: 10.1177/104063879200400108.
Congenital dyserythropoiesis with dyskeratosis is a slow, progressive, and often fatal disease in Polled Hereford calves. Affected calves have a macrocytic normochromic anemia with a mild reticulocytosis. Studies indicate that calves are hyperferremic with increased saturation of serum total iron binding capacity, which rules out iron deficiency as a cause. Other secondary causes of dyserythropoiesis, including cobalamin and folate deficiencies, are unlikely because serum cobalamin and folate levels of affected calves were normal. Virus isolation was negative, and failure to identify bovine retroviral antigens or antibodies from several calves suggested that viral agents were not involved. Bone marrow cytologic findings were similar to those in congenital hereditary dyserythropoiesis in humans and included occasional multinucleate cells, internuclear chromatin bridging between nuclei of partially divided cells, and, more frequently, irregular nuclear shapes and chromatin patterns. DNA content and cell cycle distribution of erythroid cells appeared normal, and no electrophoretic abnormalities were detected in erythrocyte membrane proteins. The Polled Hereford syndrome is similar in many ways to type I congenital dyserythropoiesis in humans and may be an appropriate biomedical model for studying erythroid proliferation during dyserythropoiesis.
先天性红细胞生成异常伴角化不良是无角海福特犊牛的一种缓慢、进行性且常致命的疾病。患病犊牛患有大细胞正色素性贫血,并伴有轻度网织红细胞增多。研究表明,犊牛高铁血症,血清总铁结合力饱和度增加,排除了缺铁作为病因。红细胞生成异常的其他继发原因,包括钴胺素和叶酸缺乏,不太可能,因为患病犊牛的血清钴胺素和叶酸水平正常。病毒分离呈阴性,从几头犊牛中未能鉴定出牛逆转录病毒抗原或抗体,提示病毒因素未参与其中。骨髓细胞学检查结果与人类先天性遗传性红细胞生成异常相似,包括偶尔出现的多核细胞、部分分裂细胞核之间的核内染色质桥接,以及更常见的不规则核形态和染色质模式。红系细胞的DNA含量和细胞周期分布似乎正常,红细胞膜蛋白未检测到电泳异常。无角海福特综合征在许多方面与人类I型先天性红细胞生成异常相似,可能是研究红细胞生成异常期间红系增殖的合适生物医学模型。