Dale D C, Hammond W P
Department of Medicine, University of Washington School of Medicine, Seattle 98195.
Blood Rev. 1988 Sep;2(3):178-85. doi: 10.1016/0268-960x(88)90023-9.
Cyclic neutropenia is a benign, hematologic disorder characterized by recurrent episodes of severe neutropenia at 21 day intervals. There are associated cyclical variations in other blood cells. Patients with this disease have malaise, stomatitis, cervical lymphadenopathy and fever during the recurrent neutropenic periods. The exact cause of cyclic neutropenia is unknown. About one third of human cases appear to be inherited in an autosomal dominant pattern. In the other cases, the disease appears to arise spontaneously with symptoms usually beginning in infancy or early childhood. In adult patients, the disease may be acquired and occur in association with a clonal proliferation of large granular lymphocytes. Clinical studies in man and investigations in grey collie dogs, which have a very similar disease, strongly suggest that cyclic neutropenia is due to an abnormality in the regulation of early hematopoietic precursor cells. Therapy for cyclic neutropenia involves local and symptomatic therapy for the recurrent mouth ulcers and pharyngitis, and antibiotics for episodes of sinusitis, pneumonia, peritonitis, or bacteremia. Therapy with glucocorticosteroids, androgens, and plasmapheresis has been efficacious in a few adult patients, but no therapy has been proven to alter the cycling of blood counts in children. Despite their repetitive illnesses, patients with cyclic neutropenia grow and develop normally. With the help of attentive physicians and dentists, their quality of life and life expectancy are good. Current research on hematopoietic growth factors offers promise of new approaches to therapy.
周期性中性粒细胞减少症是一种良性血液系统疾病，其特征为每隔21天就会出现一次严重中性粒细胞减少的反复发病情况。其他血细胞也存在相关的周期性变化。患有这种疾病的患者在中性粒细胞减少的复发期会出现不适、口腔炎、颈部淋巴结病和发热症状。周期性中性粒细胞减少症的确切病因尚不清楚。大约三分之一的人类病例似乎以常染色体显性模式遗传。在其他病例中，该疾病似乎是自发产生的，症状通常始于婴儿期或儿童早期。在成年患者中，该疾病可能是后天获得的，并且与大颗粒淋巴细胞的克隆性增殖有关。对人类的临床研究以及对患有非常相似疾病的灰色柯利犬的调查强烈表明，周期性中性粒细胞减少症是由于早期造血前体细胞调节异常所致。周期性中性粒细胞减少症的治疗包括对复发性口腔溃疡和咽炎进行局部和对症治疗，以及对鼻窦炎、肺炎、腹膜炎或菌血症发作使用抗生素。糖皮质激素、雄激素和血浆置换疗法在少数成年患者中已显示有效，但尚无治疗方法被证明能改变儿童的血细胞计数周期。尽管患有反复性疾病，但周期性中性粒细胞减少症患者生长发育正常。在细心的内科医生和牙医的帮助下，他们的生活质量和预期寿命良好。目前对造血生长因子的研究为新的治疗方法带来了希望。
