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目的提高对首发为中枢神经系统锥体外系表现的棘红细胞增多症的临床表现、诊断与治疗方法及细胞学病理学特征的认识及鉴别。方法报道2例棘红细胞增多症患者的临床表现、辅助检查及诊治过程。结果 2例患者为青年兄妹,慢性病程,逐渐加重,主要表现均为舞蹈样不自主运动,血常规均可见红细胞形态异常,进一步行血液细胞学染色,光镜下可见棘形红细胞增多,且随病程增长,畸形红细胞比例逐渐增多,临床症状可能与棘红细胞比例有关。该病例具有家族性,兄妹血液细胞病理学特征及临床表现均类似。结论对于年轻舞蹈样锥体外系病变,需考虑棘红细胞增多症可能,及时行血液细胞学形态检查可迅速确诊。目前此病尚无有效治疗手段,总体预后不佳。
Objective To improve the understanding of the clinical manifestations, diagnosis and treatment of cytokinesis and pathological features of the first episode of extrapyramidal system of the central nervous system. Methods The clinical manifestations, auxiliary examinations and diagnosis and treatment of 2 patients with acanthosis were reported. Results The two patients were young siblings. The course of chronic disease was gradually aggravated. The main manifestations were all involuntary movements of the dance. The abnormal erythrocytes were seen in the blood and the hematopoietic cells were further stained. The number of sphenoid erythrocytes was increased under light microscope. Course of disease growth, the proportion of abnormal red blood cells gradually increased, the clinical symptoms may be related to the ratio of erythrocyte. The case has a familial, sibling blood cytopathological features and clinical manifestations are similar. Conclusion For young dance-like extrapyramidal lesions, the need to consider the possibility of anaphylaxis, prompt blood cytology examination can be quickly diagnosed. Currently there is no effective treatment for this disease and the overall prognosis is poor.