Benign familial leukopenia and neutropenia in different ethnic groups

Benign hereditary leukopenia-neutropenia has been reported in several ethnic groups, including Yemenite Jews, Blacks of South African extraction, West Indians and Arab Jordanians. The subjects with BFL were shown not to have an increased incidence of infections, and their response to infection did not differ from subjects having normal white blood cell counts. This study entails the report of two additional unrelated ethnic groups with familial neutropenia - Black Beduin and Falashah Jews. The familial nature of the phenomenon was confirmed. The suggested mechanism of this type of neutropenia is a defect in release of mature WBC from the bone marrow to the peripheral circulation. All ethnic groups thus far reported have tanned or dark skin. The significance of this common feature has still to be elucidated.     

重组人粒细胞－巨噬细胞集落刺激因子治疗肺癌化疗或放疗所致的白细胞减少

对肺癌化疗、放疗后白细胞低于３．０×１０９／Ｌ者用ｒｈＧＭ－ＣＳＦ治疗９人１７次，１０例用其他升白细胞药物者作平行对照临床试验、结果表明：ｒｈ　ＧＭ－ＣＳＦ对肺癌化、放疗所致的白细胞（粒细胞）减少的疗效显著而迅速，总有效率达１００％，用药组使白细胞恢复至正常的中位数时间比对照组缩短约１０ｄ，且有良好的可耐受性、是肿瘤化疗、放疗的有力辅助药物＊Ｐ＜０．０１。下降水平与用药组均具可比性。（３）ｒｈＧＭ－ＣＳＦ：１５０μｇ／支与３００μｇ／支。方法（１）用法：化疗或放疗后外周血白细胞总数≤３．０×１０９／Ｌ时，应用ｒｈＧＭ－ＣＳＦ３００μｇ／ｄｓｃ３－７６或１５０μｇ／ｄｓｃ７－１３ｄ。本组剂量范围１．９－５．９μｇ／ｋｇ，ｑｄ。（２）疗效判断标准：用药后外周血白细胞总数升至４．０×１０９／Ｌ以上者为有效。疗效分级为：用药≤５ｄ白细胞总数升至正常水平者为显效：用药６－１０ｄ白细胞总数升至正常水平者为有效；用＞１０ｄ而白细胞计数未升高者为无效。（３）统计方法：均数的ｔ检验。结果疗效按放疗标准判断，显效１２例次（７１％），有效５例次（２９％），总有效率为１００％。用ｒｈＧＭ－ＣＳＦ前后白细胞总数的平均值分别为（２．６?     

Rapid accurate absolute granulocyte count determination during severe leukopenia using the flow cytometer

The absolute granulocyte count (AGC) in 125 blood samples from patients with total white blood cell counts of less than 1,000/microliter was estimated using three different methods, which were then compared for efficiency and accuracy. The three methods were 25 cell differential counts using Wright's-stained blood smears, granulocyte percentage estimates from WBC counting chambers, and combined narrow- and wide-angle light-scatter characteristics determined on a flow cytometer. A survey of clinical laboratories at University Hospital Cancer Centers revealed that the smear differential was the most-often-used method in those laboratories even when less than 25 cells could be counted. Consequently the data obtained from the counting chamber and flow cytometer methods were compared to the smear differential "standard" using linear regression, and outliers were identified. There was good correlation between AGC determined by smear differential and WBC counting chamber (correlation coefficient .911) and excellent correlation between the AGC determined by smear differential and the flow cytometer method (correlation coefficient .970). The flow cytometer method used in this investigation required minimal specimen preparation, and test results were available at a rate of 60 seconds/sample. The ease of sample preparation, speed, and statistical reliability of test results makes the flow cytometer an attractive alternate method of determining granulocyte counts on leukopenic patients as compared to the stained blood smear differential.     

硫普罗宁对环磷酰胺所致小鼠白细胞减少的防治作用

目的 :观察硫普罗宁 (Tiopronin ,MPG)对化疗所致小鼠白细胞减少的预防和治疗作用。方法 :采用环磷酰胺 (CPA)造成小鼠外周血白细胞减少的动物模型 ,观察MPG对由化疗所致白细胞减少的防治作用。结果 :预先用不同剂量MPG给小鼠经口灌胃或腹腔注射 (ig或ip) ,用药 5d ,再腹腔注射 1次CPA ,然后于第 3d计数白细胞总数 ,同时与单用CPA相比差异具有显著性 (P <0 .0 5或P <0 .0 1)。先腹腔注射CPA ,再用不同剂量MPG对小鼠经口灌胃或腹腔注射进行治疗 ,然后于第 3d、第 5d计数白细胞总数 ,与单用CPA组相比差异具有显著性 (P <0 .0 5或P <0 .0 1)。结论 :MPG具有明显的预防和治疗CPA所致的小鼠白细胞减少的作用。     

国内氯氮平引起白细胞减少和粒细胞缺乏文献回顾

目的 :了解常用抗精神病药氯氮平在我国应用引起白细胞减少和粒细胞缺乏的情况。方法 :依托北京 7家图书馆 ,对《中华 (神经 )精神科杂志》等 9种精神科杂志 ,《(中国 )新药与临床杂志》等 3种药学杂志逐年进行手工检索 ,收集国内自 1979年至 2 0 0 2年 11月公开发表的有关氯氮平引起白细胞减少和 或粒细胞缺乏的文献 10 7篇 ,其中个案报道 39篇 ,进行整理分析。结果 :白细胞减少发生率为 3 92 % (12 4 3 3174 9) ,粒细胞缺乏的发生率为 0 2 1% (92 4 330 2 )。收集到的 6 2例个案报道中 ,死亡 2 1例 (33 87% ) ,粒细胞缺乏发生者的平均年龄 (34 12± 12 39)岁 ,平均剂量 (318 33± 14 5 81)mg d。在 4 7例有发生距用药时间记录的病例中 ,4 2例发生在 90天内 (89 36 % ) ,2例的应用时间分别长达 5年半和 10年 ,由其余 4 5例得到的平均发生时间为 (4 6 4 7±2 6 4 0 )天。 6 2例中有 7例指明了与其他精神药物联用 ,4例死亡 (5 7 14 % )。结论 :应用氯氮平发生白细胞减少和粒细胞缺乏的风险较高 ,用药前 3个月是粒细胞缺乏的相对高发期 ,长期应用或联用其他精神药物仍有一定风险 ,建立定期的血象监测等措施十分必要     

电超导中药经皮外治药物性白细胞减少症162例疗效观察

目的:探讨电超导中药经皮外治药物性白细胞减少症的疗效。方法:将212例患者分为2组,治疗组162例,对照组50例。治疗组用中药外治法,对照组用口服西药升白细胞治疗,10天为1疗程。2组患者均在治疗后3、7、10天进行疗效观察。结果:治疗3、7、10天后,2组疗效比较,经统计学处理,差异有非常显著性意义(P<0.01)。结论:中药外治法可作为药物性白细胞减少症的一种治疗途径应用于临床。     

RETICULAR DYSGENESIS IN A PRETERM INFANT: A Case Report

Reticular dysgenesis (RD) is a rare congenital immunodeficiency classified within the severe combined immunodeficiencies (SCIDs) and characterized by impairment of both lymphoid and myeloid cell development. Neutropenia unresponsive to recombinant human granulocyte colony-stimulating factor (rGCSF) is the hallmark of RD and the clinical course is rapidly fatal due to overwhelming infections. The authors report a female newborn at 32 weeks of gestation presenting with severe leukopenia at birth. The bone marrow showed a maturation arrest in the myeloid and lymphoid lineage. She had no response to granulocyte colony stimulating factor (rGCSF) treatment and died with sepsis at age of 2 months.     

大剂量利血生片治疗白细胞减少症的有效性和安全性

目的评价大剂量利血生片治疗白细胞减少症的临床疗效和安全性。方法将70例抗甲亢药物引起的白细胞减少症患者随机分为3组：A组30例，口服大剂量利血生片40mg／次，每日3次；B组30例，口服常规剂量利血生片20mg／次，每日3次；C组（安慰剂组）10例，口服维生素B1片20mg／次，每日3次。疗程均为4周，观察治疗前后白细胞计数的变化和不良反应，并进行组间疗效对比。结果A组显效16例，有效10例，无效4例，总有效率为87％；B组显效9例，有效12例，无效9例，总有效率为70％。两组总有效率均明显高于安慰剂组（20％，P〈0．01），但A组优于B组（P〈0．05）。在治疗过程中未发现利血生片有任何毒副作用。结论大剂量利血生片治疗白细胞减少症，疗效优于常规剂量且安全。     

驴胶补血颗粒升高白细胞作用及机制研究进展

白细胞减少症是临床常见疾病,发病原因具有多样化,最常见为药物引起,且以抗肿瘤药物引起的白细胞减少症最为广泛。对于白细胞减少症的治疗,中药能够从多角度、多层面、多靶点发挥综合调控能力。驴胶补血颗粒作为补血益气中药,具有疗效好、使用方便,易被患者接受等特点,临床已有报道其在升高白细胞方面的应用。对驴胶补血颗粒全方、药对及单味药的升高白细胞的作用及机制进行综述,为扩大该药物的临床适应症及合理使用提供参考依据。