Yang T J
Department of Pathobiology, University of Connecticut, Storrs 06268.
In Vivo. 1987 Sep-Oct;1(5):297-302.
Canine cyclic hematopoiesis (CH) was first described in Gray Collies as the lethal gray syndrome, and was subsequently shown to be a counterpart of human cyclic neutropenia (CN). The disease is characterized by a recurrent cyclic change in the levels of neutrophils and other blood elements at approximately 12-day intervals. It is caused by an autosomally recessive gene with pleiotropic effects or a CH gene which is closely linked to a gray color gene. The infectious insult on affected animals is periodic but its clinical and pathologic effects are continual and cumulative Affected dogs die after weaning and rarely survive over 6 months of age. There is evidence of immunoregulatory defects in these dogs. Reciprocal bone marrow transplantation indicates that the defect resides in the bone marrow, but the actual site and mechanism of the defect has not been established. The disease in Gray Collies represents a unique model system for studying the mechanism of cyclic hematopoiesis and hematopoietic regulation. Studies of the disease have made conceptual contributions toward understanding and treatment of human cyclic neutropenia.
犬循环性造血（CH）最初在格雷牧羊犬中被描述为致死性灰色综合征，随后被证明是人类循环性中性粒细胞减少症（CN）的对应病症。该疾病的特征是中性粒细胞和其他血液成分的水平每隔约12天出现周期性变化。它由具有多效性的常染色体隐性基因或与灰色基因紧密连锁的CH基因引起。对患病动物的感染性侵害是周期性的，但其临床和病理影响是持续且累积的。患病犬在断奶后死亡，很少能存活超过6个月龄。有证据表明这些犬存在免疫调节缺陷。相互骨髓移植表明缺陷存在于骨髓中，但缺陷的实际部位和机制尚未确定。格雷牧羊犬中的这种疾病代表了一个用于研究循环性造血机制和造血调节的独特模型系统。对该疾病的研究为理解和治疗人类循环性中性粒细胞减少症做出了概念性贡献。
