Price T H, Lee M Y, Dale D C, Finch C A
Blood. 1979 Sep;54(3):581-94.
Quantitative studies of bone marrow neutrophil pool sizes and production rates and of blood neutrophil kinetics were performed in 16 patients with chronic neutropenia without splenomegaly. Marrow netrophil cellularity was determined from a ferrokinetic estimate of marrow normoblasts and from neutrophil-erythroid ratios determined from marrow sections. Postmitotic pool turnover was derived from the postmitotic pool size and transit time, the latter determined from 3H-thymidine neutrophil emergence time. Blood neutrophil kinetics were studied with 32P-diisopropylfluoophosphate-labeled autologous neutrophils. Mitotic pool size was basal or below basal in 12 of the 16 patients. The turnover of the post-mitotic neutrophils was subbasal in 6, basal in 7, and above basal in 3 patients. Blood neutrophil turnover was within the normal range in 8 patients and decreased in 8. The degree of ineffective granulocytopoiesis was assessed by comparing the relative size of the mitotic pool, postmitotic pool turnover, and blood turnover. On this basis, 13 of the 16 patients showed appreciable degrees of ineffective granulocytopoiesis. Ineffective neutrophil production occurred both early and late in neutrophil development. These studies indicate that most patients with chronic neutropenia without splenomegaly lack a proliferative marrow response to the neutropenia and suggest that ineffective granulocytopoiesis is a common feature of this disorder.
对16例无脾肿大的慢性中性粒细胞减少症患者进行了骨髓中性粒细胞池大小、生成率及血液中性粒细胞动力学的定量研究。通过对骨髓成红细胞的铁动力学估计以及从骨髓切片确定的中性粒细胞与红细胞比例来测定骨髓中性粒细胞细胞密度。有丝分裂后池周转率由有丝分裂后池大小和转运时间得出,后者由3H-胸腺嘧啶核苷中性粒细胞出现时间确定。用32P-二异丙基氟磷酸标记的自体中性粒细胞研究血液中性粒细胞动力学。16例患者中有12例的有丝分裂池大小处于基础水平或低于基础水平。6例患者有丝分裂后中性粒细胞的周转率低于基础水平,7例为基础水平,3例高于基础水平。8例患者的血液中性粒细胞周转率在正常范围内,8例降低。通过比较有丝分裂池的相对大小、有丝分裂后池周转率和血液周转率来评估无效粒细胞生成的程度。在此基础上,16例患者中有13例表现出明显程度的无效粒细胞生成。无效中性粒细胞生成在中性粒细胞发育的早期和晚期均有发生。这些研究表明,大多数无脾肿大的慢性中性粒细胞减少症患者缺乏对中性粒细胞减少症的增殖性骨髓反应,并提示无效粒细胞生成是该疾病的一个常见特征。