2004年美国血液学恶性疾病治疗研究进展概要

第46届美国血液学会（ASH）有关血液疾病的治疗学进展主要集中在靶向治疗，如Oblimersen、利妥昔单抗＋化疗、蛋白酶体抑制剂Bortezomid、Flavorpiridol细胞周期依赖酶（CDK）抑制剂以及放射免疫治疗在血液学恶性疾病中的应用。造血干细胞移植（HCT）仍然是治疗血液学恶性疾病的有效手段，非清髓性移植最大的优点为降低移植相关病死率，这是目前的研究方向。     

血液分析仪用校准物和质控物标准解读

通过对血液分析仪用校准物和质控物性能要求进行解释和分析,尤其是对校准物和质控物的计量学溯源性的认真分析,明确了医疗器械技术审评人员针对此类产品的审评重点,更好控制产品质量。     

Pulmonary hypertension and cor pulmonale in the sickle hemoglobinopathies

Although pulmonary hypertension is frequently mentioned as a complication of the sicklemic state, careful review of the medical literature revealed only a single subject in whom cardiac catheterization data substantiated this diagnosis. In two additional patients, both clinical and autopsy findings of pulmonary vascular disease and cor pulmonale were described, although no hemodynamic studies had been performed. We have therefore detailed the clinical history, cardiac catheterization results, and autopsy findings in three previously undescribed patients. These three patients, along with the three case reports culled from the medical literature, form the substance of this review. Pulmonary hypertension should be suspected in patients with sickle hemoglobinopathy in whom either fixed dyspnea or unexplained syncope develops. Early in the course of the disease, right heart catheterization remains the only way to establish the diagnosis with certainty. Noninvasive studies such as chest x-ray, electrocardiography, and echocardiography tend to be nondiagnostic until late in the course of right ventricular failure. Although specific therapy has yet to be defined, the ominous prognosis of this complication of sickle hemoglobinopathy supports the application of experimental modalities such as continuous oxygen therapy, partial exchange transfusion, or even limited phlebotomy.     

Are Standard Human Coagulation Tests Suitable in Pigs and Calves During Extracorporeal Circulation?

The thrombogenicity of membrane oxygenators as well as clotting parameters profiles, using standard human clotting tests, was analyzed in calves and pigs during 6 h perfusion. Three calves and 3 pigs were connected to extracorporeal circulation with standard heparinization. Blood samples were taken for coagulation variables throughout perfusion, and oxygenators were examined for clot deposits at the end of the experiment. Two out of 3 oxygenators of the calf group presented clot deposits while none in the pig group did. Baseline coagulation variables of pigs showed values similar to those of humans while neither extrinsic nor intrinsic pathways could be activated in calves with standard human coagulation tests. The calf model, in conclusion, was confirmed to be a difficult model for the testing of extracorporeal circulation device resistance to thrombus formation, which is, however, not reflected by standard human coagulation tests. The pig model is a better model in which both coagulation pathways could be activated with standard human coagulation tests.     

Usefulness of bone marrow imaging in childhood malignancies

Two hundred six technetium-99m sulfur colloid bone marrow scans in 110 pediatrics patients were reviewed. The normal distribution of sulfur colloid in the lower extremities in various age groups was established. There was progressive loss of uptake with increasing age from less than two years to greater than ten years. Tumor replacement was seen as regions of decreased radioactivity, and the extent of the scan defect paralleled the response of the disease to therapy. Both chemotherapy and irradiation resulted in an extension of the Tc-99m SC to peripheral marrow sites. In irradiated areas, marrow scan defects were demonstrated and generally recovered normal activity by six months after the completion of therapy. Marrow scan abnormalities caused by tumor replacement were present in four patients despite normal bone scans and radiographs. Ultimate confirmation of tumor involvement was by needle aspiration or biopsy. Persistent marrow defects were seen in two patients with neuroblastoma who had remission of their disease: biopsy revealed myelofibrosis. Technetium-99m sulfur colloid bone marrow scanning is a sensitive monitor of altered marrow activity associated with pediatric hematologic or oncologic diseases.     

CELL-DYN3700血液分析仪的应用评价

目的对CELL-DYN3700(CD3700)血液分析仪的性能进行评价。方法参照美国临床实验室标准化委员会(NCCLS)和国际血液学标准化委员会(ICSH)文件的评价方法,对仪器的精密度、携带污染率、线性及白细胞分类等性能指标进行评价。结果CD3700血液分析仪的精密度和携带污染率都小于仪器设置的要求,线性范围能够较好地满足临床的需要,对白细胞分类结果与人工法结果有较好的相关性。结论CD3700血液分析的性能指标良好,可满足临床需要。     

血液分析仪XE-2100血小板计数准确性与血涂片复检的比较分析

目的分析血液分析仪XE-2100血小板计数的准确性和血涂片复检的关系。方法测定血液分析仪XE-2100、CD-3700和手工法血小板计数精密度、线性和相关性；选择仪器CD-3700随机检测有报警的标本用XE-2100和手工法计数血小板；比较仪器血小板计数在不同范围内的标本例数与标本血片血小板数量估计值例数的符合率。结合观察血涂片红细胞、白细胞形态。与血液分析仪血小板计数报警进行对照比较。结果XE-2100血小板计数精密度较高，变异系数〈3％，血小板计数有良好的线性。其PUI-O法和PLT-I法血小板计数与手工法血小板计数相关性为γ=0.9。标本血小板浓度越低。仪器血小板计数与血片血小板估计值符合率越低。XE-2100血小板计数报警“血小板凝集”、“PLT分布异常”分别与血片上“大血小板”、“红细胞碎片”、“小红细胞增多”的例数相关。血小板计数在正常范围（100-300）×10^9／L时，仪器PLT-O法和PLT-I法血小板计数的报警与血片上所见血小板形态最为一致。结论XE-2100血小板计数准确性较高：但当血小板浓度异常、特别是浓度减低时，仪器计数结果需结合散点图、报警内容进行血片显微镜复查。