噬血细胞综合征骨髓象特点及检验结果分析

目的探讨噬血细胞综合征的骨髓像特点及检验结果分析.方法回顾分析26例噬血细胞综合征的骨髓像特点及检验结果.结果16例(61.5%)与感染相关;以病毒感染最多见.骨髓像特点:HSP早期骨髓增生活或增生减低,粒红比减低,重症HPS骨髓增生重度减低,粒、红、巨三系减少,全部骨髓片找到吞噬了完整形态、数量不等的有核细胞、红细胞或血小板的噬血细胞.90%以上患者外周血出现白细胞和血小板二系或三系减低.90%以上患者TG高、SF高、Fib低和APTT延长.结论骨髓像找到吞噬了完整形态、数量不等的有核细胞、红细胞或血小板的噬血细胞为HPS诊断依据,HPS患者出现TG升高、SF升高,FIB减低和APTT时间延长,临床上有上述情况,必须警惕HPS发生.     

噬血细胞综合征早期实验室指标改变研究

目的探讨HPS早期实验室指标变化规律，提供HPS早期实验室诊断依据。方法2003年1月至2007年9月临床疑为HPS患者51例进行实验室检指标观察．并结合临床症状和实验室指标变化对HPS进行分期。结果符合HPS者30例，诊断为HPS早期21例；HPS早期有持续高热．峰值≥38．5℃、发热期≥4天，发病与感染相关；以病毒感染最多见，生化指标TG增高、SF增高；外周血细胞一系或二系减低；骨髓增生活跃或明显活跃，骨髓片（100％）找到吞噬了完整形态的有核细胞、成熟红细胞或血小板的噬血细胞，骨髓噬血细胞0．5～11个／ul，中位数4．2个／ul，吞噬细胞体积偏小，多为单个核，核偏一侧，可呈印戒样，胞浆灰兰或淡红色，细胞边缘不整齐。结论临床患者持续4天以上，峰值≥38．5℃，外周血白细胞和血小板一系或二系减低等，应常规作骨髓细胞学检查，以便早期发现HPS和尽早实施治疗．     

噬血细胞综合征早期实验室指标改变研究

目的探讨HPS早期实验室指标变化规律，提供HPS早期实验室诊断依据。方法2003年1月至2007年9月临床疑为HPS患者51例进行实验室检指标观察．并结合临床症状和实验室指标变化对HPS进行分期。结果符合HPS者30例，诊断为HPS早期21例；HPS早期有持续高热．峰值≥38．5℃、发热期≥4天，发病与感染相关；以病毒感染最多见，生化指标TG增高、SF增高；外周血细胞一系或二系减低；骨髓增生活跃或明显活跃，骨髓片（100％）找到吞噬了完整形态的有核细胞、成熟红细胞或血小板的噬血细胞，骨髓噬血细胞0．5～11个／ul，中位数4．2个／ul，吞噬细胞体积偏小，多为单个核，核偏一侧，可呈印戒样，胞浆灰兰或淡红色，细胞边缘不整齐。结论临床患者持续4天以上，峰值≥38．5℃，外周血白细胞和血小板一系或二系减低等，应常规作骨髓细胞学检查，以便早期发现HPS和尽早实施治疗．     

噬血细胞增生症37例报告

噬血细胞增生症,又称噬血细胞综合征.是一种从骨髓细胞角度提出的病症.可由感染、肿瘤、免疫、遗传、胶原性疾病等多种病因诱发。表现为组织细胞增生,并以活跃的吞噬表现的组织细胞为主,可吞噬红细胞、白细胞、血小板.临床有高热、肝、脾、淋巴结肿大,一系或多系血细胞减少.其特点是骨髓中出现吞噬亢进的噬血细胞.作者收治37例噬血细胞增生症,分析报告如下。     

表现为急性肝衰竭的嗜血细胞综合征二例误诊分析

目的探讨嗜血细胞综合征(hemophagocytic syndrome,HPS)的误诊原因及鉴别诊断要点。方法回顾分析我院近期收治2例HPS的临床资料。结果 2例高热均超过1周,皮肤重度黄染,肝、脾大,门诊诊断为急性肝衰竭。入院后进一步完善血常规检查发现血细胞三系均明显减低,遂行骨髓穿刺细胞学检查,例1报告为骨髓有核细胞增生减低,红系比例增高,噬血组织细胞占有核细胞的3%,噬血细胞吞噬血小板多见;例2表现为增生性骨髓象,粒、巨二系成熟障碍,未见明显异常细胞。均确诊为HPS,例1抢救无效死亡,例2经积极吸氧、抗感染、糖皮质激素、营养支持、对症治疗10 d,病情无明显好转,自动出院。结论 HPS临床较少见,早期症状、体征无特异性,可以急性肝衰竭为主要表现,提高对本病的认识,及时行骨髓细胞学检查可尽早明确诊断。     

噬血细胞综合征实验室诊断和临床分析

目的总结噬血细胞综合征（HPS）的临床特征,探讨实验室诊断指标,特剐是细胞形态学对噬血细胞综合症的诊断价值。方法对28例HPS患者的临床特征、实验室诊断结果、形态学进行回顾性分析。结果HPS患者常以发热、肝脾淋巴结肿大、血细胞减少为主要表现,骨髓组织细胞增多,100％见到噬血细胞,伴有不同程度的肝功能损害、凝血功能异常、三酰甘油（TG）、乳酸脱氢酶（LDH）血清铁蛋白（SF）升高,纤维蛋白原（FIB）降低。结论HPS病情凶险,进展迅速,外周血细胞减少、骨髓涂片中噬血细胞≥1％,高TG、高LDH、高SF及低FIB都是诊断HIS的重要指标,通过对HPS实验室特征的认识,有利于HPS的早诊断、早治疗、减少死亡率。     

感染相关性噬血细胞综合征早期骨髓细胞学等指标探讨

目的探讨感染相关性噬血细胞综合征（IAHS）的早期骨髓细胞学特点及常用实验指标变化的临床意义。方法对51例IAHS患者的临床特征、骨髓细胞形态学、外周血细胞、DIC、生化及血清铁蛋白等进行动态综合分析。结果IAHS患者起病急骤,临床表现缺乏特异性。常以高热、进行性衰竭和肝脾淋巴结肿大为临床特点。血细胞单系或二系、三系进行性下降。骨髓涂片中可见数量不一的吞噬型组织细胞,找到吞噬完整的红细胞及吞噬多个血小板的吞噬细胞具有重要价值,主要特点是成熟的组织细胞件有活跃的吞噬血细胞现象。而出现较多吞噬细胞,胞浆内出现大量空泡且见有吞噬多个红细胞或多个血小板现象时,提示患者病情危重。血清铁蛋白升高,早期甘油三酯正常或升高,血清乳酸脱氢酶及谷丙、谷草转氨酶升高。纤维蛋白原正常或降低,碱性磷酸酶染色积分增高或正常。结论持续或间歇性高热,血清铁蛋白增高,外周血细胞进行性下降,乳酸脱氢酶升高,骨髓涂片中可见数量不一的吞噬型组织细胞,找到吞噬完整的红细胞及吞噬多个血小板的吞噬细胞,可作为1AHS早期初步诊断依据;可为IAHS的早期治疗,改善预后提供证据。     

噬血细胞综合征和恶性组织细胞病细胞形态学分析

目的 探讨细胞形态学在诊断噬血细胞综合征(HPS)和恶性组织细胞病(MH)中的价值.方法 对78例HPS和61例MH患者的骨髓片、外周血片、外周血液浓缩片中的细胞形态学进行了详细观察,并结合外周血碱性磷酸酶染色(NAP)、血清铁蛋白及纤维蛋白原等的结果 进行了综合分析.结果 HPS与MH两者的细胞形态、数量有明显韵差异,HPS主要特点是成熟的组织细胞伴有活跃的吞噬各种血细胞现象,可见异常组织细胞.NAP积分增高或正常,血清铁蛋白(SF)及甘油三酯(TG)升高;纤维蛋白原(FIB)降低.MH主要特点是异常组织细胞恶性增殖,可见吞噬型组织细胞.异常组织细胞和多核巨组织细胞是诊断要点,NAP积分明显减低.结论 细胞形态学检查是诊断HPS及MH的主要方法 ,具有重要的临床意义.     

35例继发性噬血细胞综合征血象、骨髓象结果分析

目的为进一步认识与重视继发性噬血细胞综合征（HPS）临床表现和血细胞变化特点。方法采集患者外周血和骨髓穿刺细胞涂片,经瑞-姬氏染色在普通光学显微镜下观察骨髓片2张、外周血片2张和经ACD抗凝的外周血浓缩涂片2张,进行细胞形态学观察分析,并进行碱性磷酸酶染色观察计算阳性率及积分值;比较分析部分患者治疗前后的血象和骨髓象变化。结果35例HPS患者因肺部感染24例（67％）;寄生虫感染1例（3％）;结缔组织病2例（6％）;急性支气管炎、扁桃体炎等7例（20％）;经病理细胞学确诊为恶性淋巴瘤1例（3％）。所有病例均有不同程度的发热,伴全血细胞减少者15例（43％）,影响二系以上血细胞减低者20例（57％）;本组患者骨髓涂片均出现成熟的组织细胞良性增生伴有活跃的吞噬各种血细胞现象,组织细胞数占骨髓有核细胞0．02～0．18。其中有9例患者病情好转复查血象、骨髓象正常,骨髓涂片未见噬血型组织细胞,比较分析治疗前后的检测结果,差异均有统计学意义（P〈0．01）。外周血浓缩涂片均未找到异常组织细胞,仅1例可见成熟单核样组织细胞（3％）;末梢血片碱性磷酸酶（NAP）染色积分值增高（〉80分）有25例,积分正常有9例,1例积分减低（〈35分）。结论诊断HPS的关键是骨髓细胞学检查,检验人员对此病应有充分认识。要正确识别骨髓涂片中噬血细胞,并根据其中组织细胞的成熟程度、噬血的活跃程度及转规情况作为诊断和鉴别诊断HPS与恶性组织细胞病（MH）的重要依据;而外周血浓缩涂片细胞形态学分析和NAP染色亦可作为HPS和MH诊断与鉴别诊断的辅助指标。     

巨噬细胞作用增强引起脓毒症时血红素加氧酶-1表达增高

巨噬细胞功能失控导致其吞噬血细胞作用增强是噬血细胞综合征（HPS）的一个典型特征。最近瑞典学者报告在脓毒症时也观察到了同样的现象．并且吞噬血细胞作用增强还促使具有抗炎作用的血红素加氧酶-1（HO-1）释放显著增多。研究者采集了28例死于脓毒症患者和8名健康志愿者（对照）的骨髓标本，观察骨髓象中巨噬细胞吞噬红细胞的现象，并检测CD163、HO-1、铁蛋白、CD8和粒酶B的表达。结果显示．脓毒症患者骨髓的所有检测指标均显著高于对照组，骨髓象中巨噬细胞吞噬红细胞现象十分活跃．这可能是巨噬细胞HO-1表达增高的一个促进因素。由此研究者认为，脓毒症与HPS时噬血巨噬细胞活性增高的机制相似，也提示巨噬细胞吞噬红细胞作用增强引起HO-1表达增高可能对脓毒症时炎症反应起抑制作用。     

Inhibition of the activation of Hageman factor (factor XII) by peripheral blood cells.

Suspensions of peripheral blood mononuclear cells (PBMC), monocytes, T or B lymphocytes, platelets or granulocytes, and cell-depleted supernatant fluids of these suspensions inhibited activation of Hageman factor (HF, Factor XII) by ellagic acid, a property not shared by erythrocytes. PBMC also inhibited HF activation by glass or sulfatides. Contaminating platelets may have contributed to inhibition by PBMC. Elaboration of agents inhibiting HF activation required metabolically active cells. The inhibitor(s) in PBMC supernates were not identified with known agents, but had properties of a nonenzymatic protein. PBMC supernates did not contain fibrinogen, nor alter the thrombin, prothrombin, or partial thromboplastin times of normal plasma, amidolysis by activated plasma thromboplastin antecedent (Factor XIa) or activated Stuart factor (Factor Xa) or esterolysis by C1 (C1 esterase); they inhibited plasmin minimally. These experiments suggest that peripheral blood cells may impede intravascular coagulation. Whether this property helps maintain the fluidity of blood is unclear.     

82例儿童噬血细胞综合征检验指标分析及成分输血治疗

目的探讨噬血细胞综合征患儿检验指标变化及成分输血在该病支持治疗中的作用。方法将符合噬血细胞综合征诊断条件的患儿分为输血组和未输血组,统计分析患儿病原体检测结果,分别比较其白细胞计数、血红蛋白、血小板计数、丙氨酸氨基转移酶、单纯天冬氨酸转移酶、乳酸脱氢酶、甘油三酯、纤维蛋白原、部分凝血活酶时间及C-反应蛋白等结果,统计输血组不同ABO血型患儿输注成分血的品种和数量。结果在78.05%(64/82)的患儿体内检出病原体,其中巨细胞病毒感染占大多数(73.44%,47/64)。2组患儿的多项检验指标均有异常,输血组患儿的血红蛋白水平、血小板计数、纤维蛋白原含量、部分凝血活酶时间等检验指标较未输血组的差异有统计学意义(P<0.05)。其他检验指标的差异无统计学意义(P>0.05)。输血组患儿输注最多的血液成分是新鲜冰冻血浆(人均700ml),其次是悬浮红细胞(人均4.44U)。结论巨细胞病毒感染是导致噬血细胞综合征患儿发病的主要诱因,该病患儿为多脏器受累,多种检验指标严重异常。成分输血,尤其是新鲜冰冻血浆的输注在重症噬血细胞综合征患儿支持治疗中发挥了重要作用。     

Hemostatic parameters and platelet activation by flow-cytometry in normal pregnancy: a longitudinal study

Summary Nineteen pregnant women with uncomplicated pregnancies were studied during the first, second, and third trimesters. We measured the following hemostatic parameters: prothrombin time, activated partial thromboplastin time, fibrinogen, antithrombin III, protein C, protein S, platelet number and volume. Platelet function was examined by a cytofluorimetric method, using an anti-GPM-140 antibody which is directed against a platelet α granule membrane protein. Activated platelets were expressed as a percentage of the GMP-140-positive platelets over total platelets. Fibrinogen levels showed a steady increase during pregnancy; conversely prothrombin time, activated partial thromboplastin time, protein C, and antithrombin III showed no significant modifications and remained within the reference range. There was a decrease of protein S activity throughout pregnancy, although protein S antigen did not follow this trend. The decrease occurred early in pregnancy and persisted during the second and third trimesters, reaching a stable plateau. We observed no platelet volume change or activation: the percentage of activated platelets was within the normal reference range, even in late pregnancy.     

骨髓转移癌细胞形态学分析

目的探讨骨髓转移癌细胞形态学特征及临床表现。方法对18例骨髓转移癌患者的临床表现及血象、骨髓象进行分析。结果血常规检查白细胞(WBC)降低者占61.0%;升高者占5.5%;血红蛋白(Hb)降低者占83.3%;血小板(PLT)降低者占66.7%;全血细胞均降低者占44.4%;外周血涂片出现幼稚红细胞者占77.8%;出现幼稚粒细胞者占55.6%;红细胞沉降率升高者占77.8%。18例骨髓片中均可找到数量不等单个出现或成团、成簇的转移癌细胞。结论骨髓细胞学检查是骨髓转移癌的简单而有效的诊断方法,有助于明确或未明确诊断的恶性肿瘤患者的治疗以及延缓病情发展。     

Studies of activated GPIIb/IIIa receptors on the luminal surface of adherent platelets. Paradoxical loss of luminal receptors when platelets adhere to high density fibrinogen.

The accessibility of activated GPIIb/IIIa receptors on the luminal surface of platelets adherent to damaged blood vessels or atherosclerotic plaques is likely to play a crucial role in subsequent platelet recruitment. To define better the factors involved in this process, we developed a functional assay to assess the presence of activated, luminal GPIIb/IIIa receptors, based on their ability to bind erythrocytes containing a high density of covalently coupled RGD-containing peptides (thromboerythrocytes). Platelets readily adhered to wells coated with purified type I rat skin collagen and the adherent platelets bound a dense lawn of thromboerythrocytes. With fibrinogen-coated wells, platelet adhesion increased as the fibrinogen-coating concentration increased, reaching a plateau at about 11 micrograms/ml. Thromboerythrocyte binding to the platelets adherent to fibrinogen showed a paradoxical response, increasing at fibrinogen coating concentrations up to approximately 4-6 micrograms/ml and then dramatically decreasing at higher fibrinogen-coating concentrations. Scanning electron microscopy demonstrated that the morphology of platelets adherent to collagen was similar to that of platelets adherent to low density fibrinogen, with extensive filopodia formation and ruffling. In contrast, platelets adherent to high density fibrinogen showed a bland, flattened appearance. Immunogold staining of GPIIb/IIIa receptors demonstrated concentration of the receptors on the filopodia, and depletion of receptors on the flattened portion of the platelets. Thus, there is a paradoxical loss of accessible, activated GPIIb/IIIa receptors on the luminal surface of platelets adherent to high density fibrinogen. Two factors may contribute to this result: engagement of GPIIb/IIIa receptors with fibrinogen on the abluminal surface leading to the loss of luminal receptors, and loss of luminal filopodia that interact with thromboerythrocytes. These data provide insight into the differences in thrombogenicity between surfaces, and may provide a mechanism for purposefully passivating platelet-reactive artificial surfaces.     

低分子肝素对原发性肾病综合征的辅助治疗作用

目的　观察低分子肝素辅助治疗原发性肾病综合征的疗效及安全性。方法　对照组常规应用强的松及其它药物 ,治疗组在对照组用药的基础上加用低分子肝素 (4 0 0 0IU)腹部皮下注射 ,两组治疗前后分别检测 2 4小时尿蛋白、血浆白蛋白、总胆固醇、甘油三酯、血液流变学、凝血酶原时间 (PT)、部分凝血活酶时间 (APTT)、凝血酶时间 (TT)、纤维蛋白原 (FIB)、血小板计数 ,并观察临床症状改善情况及有无出血倾向。结果　经 4周治疗 ,两组患者临床症状均减轻或消失 ,治疗组总有效率 (96 .6 % )高于对照组 (89.3% )。治疗后两组患者尿蛋白减少 ,血浆白蛋白升高 ,总胆固醇、甘油三酯下降 ,血液粘度降低(P <0 .0 5 ,P <0 .0 1) ;在减轻蛋白尿、升高血浆白蛋白、降低血液粘度方面 ,治疗组优于对照组 (P <0 .0 5 ,P <0 .0 1) ;治疗后两组患者的PT、TT延长 ,FIB下降 (P <0 .0 5 ,P <0 .0 1) ,但治疗组更明显 ,与对照组比较 (P <0 .0 5 ,P <0 .0 1) ,APTT延长 ,但两组间无差异 (P >0 .0 5 ) ;两组治疗后血小板数虽有下降 ,但治疗前后比较无差异 (P >0 .0 5 ) ;两组患者中无一例有出血倾向。结论　低分子肝素辅助治疗原发性肾病综合征 ,可以更好地改善患者的高凝状态 ,降低血液粘度 ,减轻蛋白尿 ,提高血浆白蛋白 ,明显地增     

D-二聚体纤维蛋白原抗凝血酶Ⅲ在冠心病诊疗中的临床应用

目的通过检测冠心病患者血浆D-二聚体(D-D)、纤维蛋白原(Fib)、抗凝血酶Ⅲ(AT-Ⅲ)等指标,观察冠心病与血栓形成的相关性。方法随机选取不同类型冠心病患者100例(冠心病组)和健康体检者100例(健康对照组),检测D-D、Fib、AT-Ⅲ、凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、三酰甘油(TG)、总胆固醇(TC)、低密度脂蛋白(LDL)及高密度脂蛋白(HDL)水平并进行分析。结果冠心病组血浆D-D、Fib、TG、TC、LDL水平分别为(1.16±2.37)μg/mL、(3.50±1.06)g/L、(2.46±0.40)mmol/L、(5.54±1.21)mmol/L、(3.21±0.55)mmol/L,健康对照组分别为(0.29±0.10)μg/mL、(2.83±0.46)g/L、(2.04±0.63)mmol/L、(4.07±0.96)mmol/L、(2.92±0.68)mmol/L;冠心病组AT-Ⅲ、PT、HDL水平分别为78.6%±5.8%、(12.70±1.27)s和(2.02±0.88)mmol/L,明显低于健康对照组的104.2%±6.0%、(13.08±0.68)s和(2.37±0.77)mmol/L,差异均有统计学意义(P<0.05)。结论 AT-Ⅲ、D-D、Fib、PT与冠心病的发生、发展密切相关,联合检测有利于冠心病的早期诊断及病情判断。     

北方地区蝮蛇咬伤致DIC样综合征的临床研究

目的：探讨北方地区蝮蛇咬伤引起DIC样综合征的临床表现及预后情况。方法：对2010-06-2011-09来本院就诊的97例蝮蛇咬伤病例进行回顾性分析,对其中确诊为DIC样综合征的9例病例进行血小板计数及凝血系统指标D-二聚体、血浆凝血酶原时间、活化部分凝血活酶时间、血浆纤维蛋白原及凝血酶时间检测及分析。结果：北方地区蝮蛇咬伤后DIC样综合征的发生率约为9.3%;9例患者分别于伤后1～8d内出现DIC现象,持续时间约2～7d,均无临床DIC表现。结论：北方地区蝮蛇咬伤可引起患者凝血及纤溶系统异常,出现DIC样综合征。     

疏血通对短暂脑缺血发作患者血脂和凝血功能的影响

目的：观察疏血通对短暂脑缺血发作患者血脂、凝血功能的影响。方法：入选短暂脑缺血发作患者98例,38例为对照组,接受常规治疗,60例为观察组,在常规治疗的基础上静脉滴注疏血通注射液,2组均观察14d。治疗前后均检测血脂及凝血功能。结果：治疗后观察组总胆固醇,甘油三酯及低密度脂蛋白胆固醇水平均较治疗前及对照组明显降低;凝血酶原时间、凝血活酶时间明显延长,纤维蛋白原含量明显下降（均P〈0.05）。临床疗效比较,观察组显效率及总有效率亦明显高于对照组（20.0%、68.3%与7.9%、34.2%,P〈0.01）。结论：疏血通注射液对短暂脑缺血发作患者的血脂、抗凝及促纤溶等有明显调节作用。     

Effects of platelets and platelet-derived material on the activated partial thromboplastin time (Cephotest) coagulation test

OBJECTIVE: The Cephotest is an activated partial thromboplastin time (APTT) test used to measure the activity of the intrinsic pathway of coagulation. To perform this test, blood is usually centrifuged to obtain plasma that is almost without erythrocytes and leucocytes and with only a minimal amount of platelets. MATERIAL AND METHODS: In the present experiments blood was centrifuged at different speeds to produce either platelet-poor plasma (PPP) or platelet-rich plasma (PRP). PPP and PRP obtained from the same whole blood samples from each of several persons were tested in pairs using the standard Cephotest reagent to observe the consequences of Cephotest being performed on plasma containing platelets. The same procedure was used with a Cephotest reagent with a reduced concentration of phosphatidylserine. In succeeding experiments the PRP was preincubated with SFLLRN or Ca-ionophore to activate the platelets, a procedure also known to produce platelet-derived microparticles. PPP and PRP were compared by thrombin time and reptilase time tests to find out at which stage platelets might influence the Cephotest. RESULTS: The results showed that the platelets did influence Cephotest when using both the regular reagent and the phospholipid-reduced agent. When using the regular reagent, PRP showed a tendency towards a longer APTT than PPP. It is suggested that this was caused by platelets consuming some of the first traces of thrombin generated. CONCLUSIONS: When using the phospholipid-reduced reagent, PRP showed a shorter APTT than PPP, probably because the platelets contributed phosphatidylserine to the system. When the platelets were activated before testing, their effects on the tests were increased. Microparticles that formed during platelet activation may have contributed to these effects.