血浆置换治疗血栓性血小板减少性紫癜的临床研究

目的 研究血浆置换(PE)治疗血栓性血小板减少性紫癜(TTP)的疗效.方法 对12例患者进行血浆置换治疗,并结合免疫抑制剂及抗血小板、改善微循环治疗.结果 治疗总有效率为91.7%(11/12),1例患者死亡.结论 血浆置换治疗结合免疫抑制剂及抗血小板、改善微循环治疗是TTP的有效治疗方法.     

Plasmapheresis in the treatment of thrombotic thrombocytopenic purpura: a report of five cases

Thrombotic thrombocytopenic purpura, an acute clinical disorder of uncertain aetiology, has previously been associated with almost 100% mortality. Dramatic response to exchange transfusion has been reported, and there is some evidence that a circulating immune complex may be involved in the pathogenesis. Therefore, it was decided to employ large-volume plasma exchange by means of a continuous-flow cell separator in the management of five patients with this disease. Three patients showed a dramatic response, which was manifested by rapid correction of the neurological deficit and of the abnormalities in laboratory findings. We conclude that the thrombotic thrombocytopenic syndrome should be regarded as an indication for emergency plasma exchange.     

9例血栓性血小板减少性紫癜的临床分析

目的分析血栓性血小板减少性紫癜（TTP）的临床特征和治疗方法选择的价值。方法对9例TTP患者的临床资料进行分析。结果9例患者6例为女性，男：女=1：3；其中系统性的红斑狼疮5例，严重感染、败血症3例，尿毒症1例。临床表现以血小板减少性出血、微血管病性溶血性贫血和神经系统症状的“三联症”起病的5例；三联症伴发热和肾脏病变“五联症”起病的2例；三联症伴发热起病2例。4例及时进行血浆置换为主治疗后均有效；其余5例未给予血浆置换的3例死亡。结论TTP多见于年轻女性，病因以系统性红斑狼疮最多见，感染次之。临床表现以三联征表现最多，以后多表现为四联征或五联征。及时给予血浆置换为主的治疗抢救成功率较高。     

Thrombotic thrombocytopenic purpura with terminal pancytopenia

A 27 year old housewife developed thrombotic thrombocytopenic purpura during the twelfth week of pregnancy. She had partial response to initial plasma infusion and subsequent plasmapheresis. However, her clinical course was complicated by the development of severe pancytopenia the consequence of a hypocellular marrow. She succumbed to septicaemic shock one month after diagnosis. The development of hypocellular marrow in thrombotic thrombocytopenic purpura has not been reported before.     

血浆置换治疗血栓性血小板减少性紫癜2例报告

对2例治血栓性血板减少性紫癜（TTP）患者每天进行血浆置换（PE）治疗，每次清除及补充1个血浆量，为40ml/kg新鲜冰冻血浆，并补充红细胞悬液，PE后临床症状迅速消失，实验室检查基本恢复正常，随访无复发，表明PE明显优于传统治疗方法，能改善TTP的预后。     

血浆置换治疗血栓性血小板减少性紫癜患者的临床分析

目的观察血浆置换治疗血栓性血小板减少性紫癜（TTP）的疗效。方法以新鲜血浆为置换液进行血浆置换。结果 7例患者中1例死亡,6例好转出院。结论血浆置换是TTP的首选治疗方法,它能够降低患者的病死率,效果肯定。     

血栓性血小板减少性紫癜——附4例报告及文献复习

目的：探讨血栓性血小板减少性紫癜（TTP）的诊断、治疗及发病机理。方法：用血浆置换治疗4例TTP,同时辅以皮质激素等治疗。结果：4例病人均获得缓解,其中1例再发后仍获缓解。结论：TTP是一种起病急且凶险的疾病,临床分为原发性及继发性两类。继发性多与妊娠、感染、某些药物、转移性肿瘤、骨髓移植等因素有关。其发病机制与内皮细胞损伤、血管性血友病因子（von willebrand factor,vWF）在血中释放增加及其在血清中裂解障碍、vWF异常大分子多聚体（ULvWF）形成有关。血置换是目前治疗本病的首选有效方法。     

血浆置换治疗血栓性血小板减少性紫癜的临床研究

目的:探讨血浆置换治疗血栓性血小板减少性紫癜(TTP)的临床疗效。方法:对14例确诊为TTP的患者给予血浆置换联合免疫抑制剂治疗。结果:14例TTP患者中,9例为特发性,5例为继发性,其临床均表现为Moschcowitz′s五联征即:血小板显著降低,微血管性溶血性贫血(MHA),神经系统症状,肾脏损害及发热。给予血浆置换及免疫抑制剂联合治疗,2例死亡,12例症状明显缓解,总有效率为85.7%。结论:血浆置换联合免疫抑制剂是治疗TTP的有效方法。     

Plasma exchange in thrombotic thrombocytopenic purpura

Three patients were recently treated for thrombotic thrombocytopenic purpura (TTP). One presented with toxic shock syndrome; TTP developed but promptly responded to a regimen of antiplatelet agents, steroids and plasma exchange. In another the manifestations of TTP developed after presentation with hypertension and abdominal pain. This patient responded to a similar regimen but required extended treatment before remission could be maintained with medications alone. In the third patient the full TTP syndrome appeared after several days of plasma exchange treatment for hemolyticuremic syndrome. He did not respond. It is suggested that TTP may present in many forms initially, that microangiopathic hemolysis may be a late manifestation and that the optimal therapy is not known.     

14例血栓性血小板减少性紫癜临床研究

目的探讨血栓性血小板减少性紫癜（TTP）患者的临床特征及治疗方案,进而提高临床TTP 患者的诊疗水平。方法对14 例TTP 患者的一般情况、临床特征、实验室检查、治疗方案、疗效及预后进行回顾性研究。结果神经精神症状有13 例,五联症表现4 例,三联症表现9 例,复发2 例,死亡6 例。血浆置换（PE）前后血小板（PLT）、血红蛋白（Hb）及乳酸脱氢酶（LDH）监测值有不同,差异有统计学意义（P <0.05）。2 例患者联合利妥昔单抗治疗,缓解率为100%（2/2）。结论TTP患者多以三联症起病,常伴随精神神经症状,血浆置换是其首选治疗方案。对复发TTP 患者,利妥昔单抗可以提高缓解率。     

Infections with Escherichia coli O157:H7 in Washington State. The first year of statewide disease surveillance

In 1987, Washington became the first state to require that infection with Escherichia coli serotype O157:H7 be reported. In the first year of surveillance, 93 cases were reported, yielding an annual incidence of 2.1 cases per 100,000 population. The median age of case patients was 14 years (range, 11 months to 78 years), with the highest attack rate among children younger than 5 years (6.1 cases per 100,000 population per year). Bloody diarrhea was present in 95% of reported cases, 12% of patients developed either hemolytic-uremic syndrome or thrombotic thrombocytopenic purpura, and one patient died. Suspected secondary cases were seen in 5% of households. Fifty-six (60%) cases occurred during June through September, as did 73% of the cases of hemolytic-uremic syndrome or thrombotic thrombocytopenic purpura. Cases reported during the summer months were more likely than cases reported at other times of the year to be in children younger than 10 years. Medications, including antimicrobial medications, did not influence the duration of symptoms, nor did they appear to alter the risk of developing hemolytic-uremic syndrome or thrombotic thrombocytopenic purpura. This newly established surveillance system in Washington demonstrates that E coli O157:H7 is an important and common cause of bloody diarrhea in the United States.     

Plasma exchange in Canada. The Canadian Apheresis Study Group.

Since 1982 the Canadian Apheresis Study Group (CASG) has collected data on plasma exchange activities in Canada. In 1987, 5907 such procedures were carried out on 700 patients for more than 22 different diseases; this represented an increase of 28% over the figure for 1982. A shift in activity has occurred over the years; originally hematologic disorders accounted for most of the procedures; however, in 1987, 60% of the exchanges were done to treat neurologic disorders, mainly myasthenia gravis and acute and chronic Guillain-Barré syndrome. Several prospective randomized clinical trials have recently been completed by the CASG in the hope of determining the optimal application of plasma exchange. These studies, currently under review, include 168 patients with multiple sclerosis, 100 with thrombotic thrombocytopenic purpura and 43 with rapidly progressive glomerulonephritis. Reactions occur in 12% of cases; they are usually minor and are limited to circumoral paresthesia, mild hypertension or hypotension and hives.     

Elevated platelet-associated IgG in thrombotic thrombocytopenic purpura

A 41-year--old woman with thrombotic thrombocytopenic purpura (TTP) was treated with prednisone and splenectomy with complete recovery from her illness. Analysis of the patient's platelets for immunoglobulins showed abnormally elevated levels in the range of those seen in thrombocytopenic patients with systemic lupus erythematosus, immune vasculitis, and infectious mononucleosis. This finding is consistent with the concept that the immune system may play a role in the pathogenesis of TTP.     

Prostacyclin and thromboxane A2 in thrombotic thrombocytopenic purpura

A study was conducted to find whether a deficiency in prostacyclin (prostaglandin I2; PGI2) is implicated in the pathogenesis of thrombotic thrombocytopenic purpura. Plasma samples from two patients with the disease before treatment and from 22 healthy controls were therefore assayed for concentrations of 6-oxo-PGF1 alpha and thromboxane B2, the stable metabolites of PGI2 and thromboxane A2, respectively. Neither of the patients responded to treatment, which in one case included an infusion of PGI2. Both patients had normal concentrations of 6-oxo-PGF1 alpha and thromboxane B2, thus implying that circulating amounts of PGI2 and thromboxane A2 were also normal. These findings suggest that 6-oxo-PGF1 alpha may be detectable in normal amounts in thrombotic thrombocytopenic purpura and that the condition need not be associated with a high concentration of thromboxane A2.     

血栓性血小板减少性紫癜6例临床分析

目的：探讨血栓性血小板减少性紫癜(TTP)的早期诊断及治疗方法,提高对本病的认识。方法：回顾性分析6例TTP患者住院病历资料。结果：6例患者均有血小板减少、徼血管病性溶血性贫血(MAHA)、明显精神神经症状。6例患者均采用血浆置换(PE),联用肾上腺糖皮质激素和抗血小板药物等综合治疗。4例存活,2例死亡。结论：TTP临...     

血栓性血小板减少性紫癜的临床诊治体会

目的 对临床病例行回顾性分析,了解血栓性血小板减少性紫癜(TTP)的临床特征及治疗方法,以加深对本病的认识,提高对TTP的诊疗水平.方法 对我院2003年5月～2010年9月诊断的8例TTP的临床表现、治疗及预后进行回顾性分析.结果 8例TTP患者中,1例为男性,7例为女性,其中1例为妊娠期者.7例临床症状缓解,外周血...     

血液细胞形态学误诊与漏诊分析

目的:通过对遗传性球形红细胞增多症、冷凝集素综合症、血栓性血小板减少性紫癜、流行性出血热、肺吸虫病、静点头孢哌酮钠饮酒后类白反应及疟疾等疾病血液细胞形态学误诊、漏诊分析,从而提高医学检验工作者对血液细胞形态学在临床工作中重要性的认识。方法:所有病例均采用不抗凝外周血做血涂片,用瑞一姬染色,通过具有细胞形态学丰富工作经验的检验人员观察红细胞、白细胞及血小板形态、数量及血液寄生虫。并进行相关的血液学实验及生化检查。结果:通过对细胞形态学认真观察和相关实验室检查及临床表现,所有病例均得到明确诊断。结论:血细胞分析仪在形态学检查方面只能是一种过筛手段,在可疑情况,尤其在病理条件下必须进显微镜及相关实验室检查,以便减少血液细胞形态学误诊与漏诊,从而能对疑难血液病做出明确诊断。     

血浆置换治疗血栓性血小板减少性紫癜的疗效观察

目的 进一步了解血浆置换治疗血栓性血小板减少性紫癜(TTP)的疗效. 方法 对4例确诊为TTP的患者给予糖皮质激素治疗联合血浆置换,深静脉置管建立体外循环,用贝朗Diapact CRRT机及费森尤斯P2S血浆分离器进行血浆置换,新鲜冰冻血浆为置换液,血浆置换量35～ 40 mL/kg,置换速度15 ～20 mL/min,血流速度120～150 mL/min,小剂量低分子肝素抗凝.血浆置换每日或隔日治疗1次.观察血液检验指标和临床症状改善情况. 结果 4例TTP患者每例完成3～5次血浆置换,治疗后血小板计数(PLT)、血红蛋白(Hb)上升,尿素(BUN)、总胆红素(IBIL)、乳酸脱氢酶(LDH)降低,治疗前后的结果经统计学处理,P＜0.01,随访6个月全部治愈.结论 血浆置换治疗TTP效果明显,TTP病人应争取早期血浆置换治疗,提高疗效,降低病死率.     

Thrombotic thrombocytopenic purpura associated with pregnancy in two sisters.

Two sisters suffered from thrombotic thrombocytopenic purpura late in their first pregnancies. HLA typing of the patients and their immediate family members demonstrated no obvious relationship. Hereditary aspects, association with pregnancy, prognosis and management of pregnant women with TTP are discussed.     

血栓性血小板减少性紫癜诊断与治疗的探讨

目的：了解血栓性血小板减少性紫癜（ＴＴＰ）临床特点,方法：对５例ＴＴＰ患的临床资料进行总结,复习献,对其临床特点。诊断和治疗进行分析。结果：５例ＴＴＰ患,均有典型五联征,经血浆置换、皮质激素、输血浆等治疗后。２例缓解,３例死亡。结论：ＴＴＰ是一种致死率高疾病,正确诊断和及时处理在临床上尤为重要。治疗方面最好采用血浆置换、输新鲜血浆,免疫抑制剂及脾切除也可应用。