目的 检测血细胞减少患者外周血红细胞和中性粒细胞细胞膜糖基磷脂酰肌醇（GPI）连接的补体调节蛋白衰变加速因子（CD55)和膜反应性溶血抑制物（CD59）表达情况，并探讨其临床意义。 方法 2006年7月－2011年3月，采用直接免疫荧光标记法流式细胞仪检测182例血细胞减少患者外周血CD55及CD59表达情况，其中阵发性睡眠性血红蛋白尿（PNH）9例，再生障碍性贫血（AA）-PNH综合征8例，AA 83例，骨髓增生异常综合征51例，自身免疫性溶血性贫血11例，造血功能停滞6例，缺铁性贫血7例，巨幼细胞性贫血4例，脾功能亢进3例。 结果 PNH及AA-PNH患者CD55、CD59抗原缺失率均较其他血细胞减少者明显增高。 结论 流式细胞仪检测外周血中红细胞和中性粒细胞膜CD55和CD59抗原表达缺失率是目前诊断PNH可靠和敏感的方法，也是对PNH、AA-PNH早期诊断敏感指标，并且PNH克隆检测还能为诊断疾病提供鉴别诊断依据。
【摘要】 目的 观察t(4; 22)致血小板源性生长因子受体α（the platelet-derived growth factor receptor alpha， PDGFRA）异常的髓系/淋巴系肿瘤的临床特点。 方法 对2010年6月收治的1例t(4; 22)致PDGFRA异常的髓系/淋巴系肿瘤患者的临床资料进行回顾性分析，并对其临床特点、实验室检查、诊断、治疗进行总结。 结果 该疾病临床表现及骨髓涂片检查类似慢性粒细胞白血病（chronic myelogenous leukemia，CML），但无CML特征性Ph染色体和（或）BCR/ABL融合基因，而细胞遗传学检测显示4号与22号染色体易位，诊断为t(4; 22)致PDGFRA异常的髓系/淋巴系肿瘤。采用羟基脲及干扰素治疗后可获得完全血液学缓解。 结论 t(4; 22)致PDGFRA异常的髓系/淋巴系肿瘤是一类罕见疾病，临床表现与CML相似，t(4; 22)及BCR/PDGFRA融合基因阳性是诊断该类疾病的关键。【Abstract】 Objective To observe the clinical features of myeloid and lymphoid neoplasms with t (4; 22) induced abnormalities of the platelet-derived growth factor receptor alpha (PDGFRA) to increase the identification and reduce the misdiagnosis. Methods The clinical data of one patient with myeloid and lymphoid neoplasm with t (4; 22) induced abnormalities of PDGFRA diagnosed in June 2010 was retrospectively analyzed. We summarized the clinical features, morphology, genetics, diagnostic criteria and therapy about this kind of disease. Results The patient had a clinical manifestation and bone marrow smear result of chronic myelogenous leukemia (CML). But the result of genetic analysis found no translocation of chromosomes 9 and 22 juxtaposing BCR and ABL gens. Cytogenetic analysis showed an abnormal karyotype with rearrangement of chromosomes 4 and 22. So the patient was diagnosed myeloid and lymphoid neoplasms with t (4; 22) induced abnormalities of PDGFRA. After receiving interferon and hydroxyurea, the patient achieved complete hematologic remission. Conclusion Myeloid and lymphoid neoplasms with t (4; 22) induced abnormalities of PDGFRA is a rare kind of disease. Its clinical feature is similar to that of CML. The key of diagnosis is genetics.
ObjectiveTo develop a method to quantitatively determine the microparticles (MP) from different sources in plasma by nine-color flow cytometry. MethodsAnnexin-V and 8 antibodies including CD235a, CD41a, CD45, CD34, CD66b, CD20, CD3 and CD14 were used to establish nine-color flow cytometric panel.Platelet poor plasma samples were single-stained and stained with 1 of 8 antibodies lacking respectively, and then we determined the detector voltages and compensations.From December 2014 to January 2015, we detected and analyzed 10 plasma samples from normal adults, and repeatability test and dilution tests were done. ResultsIn staining lacking 1 of 8 antibodies, the percentage of positive MP populations change was all less than 15% based on the population number in single-stained experiment.In dilution tests, there were good linear correlations between MPs from platelets and erythrocytes.In repeatability test, the coefficient of variation of MP from erythrocytes, platelets and granulocytes was all less than 10%.In the platelet poor plasma samples from normal adults, MP from platelets, erythrocytes, endotheliocytes, monocytes, granulocytes, B and T lymphocytes could be detected, and the average concentration of them were respectively 132.6/μL[(60.6-288.9)/μL], 35.4/μL[(22.0-99.7)/μL], 21.6/μL[(3.3-45.5)/μL], 13.9/μL[(7.3-35.1)/μL], 60.0/μL[(22.5-101.2)/μL], 21.9/μL[(6.0-33.4)/μL]and 1.2/μL[(0.7-2.8)/μL]. ConclusionsQuantitatively determining MP from different sources in plasma by nine-color flow cytometry has been successfully developed.This method is simple and fast, and can be applied in clinical detection.
目的 探讨骨髓增生异常综合征（MDS）患者的临床特点。 方法 选取我院2008年3月－2012年10月确诊为MDS的231例患者临床资料进行回顾性分析。患者年龄21～87岁，中位年龄59岁。 结果 231例患者中，难治性血细胞减少伴多系发育异常（RCMD）最多见，占45.0%（104/231）；以贫血乏力症状就诊多见占66.7%（154/231）；血常规中以全血细胞均减少多见占61%（141例/231例）；网织红细胞以正常或增高为主占61%（141/231）；低荧光值增高多见62%（144/231）。乳酸脱氢酶和铁蛋白在各诊断亚型及各国际预后积分系统（IPSS）评分间存在差异，其中乳酸脱氢酶在难治性贫血伴原始细胞增多2型（RAEB-2）中高于综合组：难治性贫血（RA）、 难治性贫血伴环状铁粒幼细胞（RAS）、5q?综合征及RCMD相比较差异有统计学意义（P＜0.05），高危组乳酸脱氢酶高于中危1组及中危2组，其差异有统计学意义（P＜0.05），高危组铁蛋白高于中危1组其差异有统计学意义（P＜0.05），其余差异无统计学意义（P＞0.05）。染色体异常率为39%，其中20例为复杂染色体核型，IPSS评分中危1最多见为52.4%（55/105）。 结论 MDS临床表现多样，缺乏特异性，需综合骨髓涂片、活检、细胞遗传学的结果提高诊断率。