以神经系统症状为首发表现的血栓性血小板减少性紫癜误诊1例

血栓性血小板减少性紫癜（TTP）是一种少见的原因未明的微血管病性溶血和血栓性微血管病。其特点是起病急、病情发展迅速、凶险、病死率极高。TTP患者有时以神经系统症状首发，易误诊为“脑炎、脑血管病”等，为提高对本病的认识，现将石家庄市第三医院收治的以神经系统症状首发的TTP1例报道如下。     

以神经系统症状为首发的血栓性血小板减少性紫癜1例诊治体会

1 病例摘要 患者,男,40岁,因"头痛20余天,加重伴阵发性意识不清1周"入院.患者20余天前感冒后开始反复出现头痛、伴有眩晕、胸闷、发热,未予特殊处理,近1周来症状发作次数增加,并伴有阵发性的短暂意识不清.体检:T 37.4℃,P 80次/分,R 20次/分,BP 130/76 mmHg,意识清楚,左侧乳头下、右侧大腿内侧可见少许散在出血点、压之不褪色.     

血栓性血小板减少性紫癜1例报告

血栓性血小板减少性紫癜１例报告广东省花都市人民医院内科（５１０８００）黄干洪血栓性血小板减少性紫癜（ＴＴＰ）甚为罕见，症状凶险，临床上易与原发性血小板减少性紫癜等疾病相混淆，最近我院成功抢救１例。现报道如下。患者女，５６岁。因皮下出血点、瘀斑伴尿黄６...     

血栓性血小板减少性紫癜一例报告

血栓性血小板减少性紫癜 (ＴＴＰ)为一种不常见的微血管病性溶血和血栓性微血管病。我院自1997年至今 ,共收治血小板减少性紫癜患者 116例 ,确诊为ＴＴＰ的仅一例 ,现报道如下。1　临床资料患者 :女 ,4 3岁 ,已婚 ,汉族 ,江苏射阳农民。因四肢抽搐伴神志不清 1小时于 2 0 0 2年 3月 9日零时入院。患者 1小时前睡眠中突然尖叫 ,口吐白沫 ,四肢抽搐 ,持续约 4～ 5分钟后自行停止 ,伴神志不清 ,呼之不应 ,小便失禁。追问病史 ,患者一周前曾有头痛 ,畏寒 ,发热 (体温未测 ) ,曾在常熟当地卫生院予补液 2天 (用药不详 ) ,发热有所好转 ,但仍有头…     

以感冒为首发症状的血栓性血小板减少性紫癜1例

患者,男,40岁,主因咳嗽、发热1周伴精神错乱、黄疸半天而于2008年12月10日21：00收入我科。患者于入院前1周出现咳嗽、发热等感冒症状,院外输液治疗（具体用药不祥）,效果不佳,半天前输液过程中突然出现精神错乱、烦躁、言语不清、答非所问。     

血栓性血小板减少性紫癜神经系统损害11例分析

现将我科2002～2005年收治的血栓性血小板减少性紫癜（thrombotic thrombocytopenic purpura,TTP）11例分析如下.     

以脑出血为首发表现的血栓性血小板减少性紫癜

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)是一种临床不常见的血栓性微血管病,有时以神经系统症状首发或因神经系统症状突出,极易误诊为脑血管疾病.我院2005～2008年收治以脑出血为首发表现的TTP患者8例,现报告如下.     

以血栓性血小板减少性紫癜为首发表现的干燥综合征1例报告及文献复习

目的;提高风湿科医生对干燥综合征合并血栓性血小板减少性紫癜的认识。方法：报告一例干燥综合征合并血栓性血小板减少性紫癜病例并复习相关文献。结果：患者为41岁女性,急性起病,以头昏、乏力、发热伴晕厥为主要临床表现,免疫指标抗核抗体、抗SSA、抗SSB、抗RNP抗体阳性,多次查血象三系均降低。血涂片发现有红细胞碎片。唾液腺检查发现异常,眼科检查发现干眼症。经血浆置换、输住新鲜冰冻血浆、糖皮质激素及免疫抑制剂长春新碱和环磷酰胺等治疗后病情好转。结论：与干燥综合征相关的TTP鲜见报道,其发病率低,死亡率高,如诊断及治疗不及时,将严重威胁病人的生命。因其发病机制、临床表现及治疗方面较复杂,应于重视。     

以脑血栓为首发的血栓性血小板减少性紫癜1例报道

患者，男，43岁．主因“发作性左侧肢体麻木无力3d，意识障碍5h”，于2003年9月19日凌晨1时入院。入院前3天患者活动中出现左侧肢体麻木无力感，约20min缓解，自觉全身乏力，纳差，腰背酸痛不适。家属发现患者颜面皮肤发黄，胸前有散在出血点。患者能坚持日常工作，未就诊治疗。此次来院5h前患者再次发作左侧肢体麻木1次，20min缓解，未诉头晕、头痛、发热、腹痛等不适，遂来医院急诊室，自行大便1次后，出现恶心，呕吐少量胃内容物，十几分钟后出现神志不清，躁动，小便失禁。     

血栓性血小板减少性紫癜1例分析

对血栓性血小板减少性紫癜(TTP)1例分析如下。1病历摘要男,75岁。因腹部不适3个月、加重1d、寒战1 h于2010-07-28T12:00入院。查体:T37.2℃,神志清晰,精神不振,贫血貌,双下肢散在新鲜出血点,浅表淋巴结未触及肿大,牙龈无增生,胸骨无压痛,双肺呼吸音低,心律规整,腹平软,剑突下压痛,无反跳痛,肝脾未触及,肝区叩痛,无移动性浊音,四肢肌力、肌张力正常,各腱反射(++),病理征(-)。     

Thrombotic thrombocytopenic purpura and pregnancy: report of four cases and literature review

Thrombotic thrombocytopenic purpura (TTP) is a severe life-threatening hematological disorder affecting the microcirculation of multiple organ systems. Infection, pregnancy, cancer, drugs, and surgery are frequently associated with the initial episodes and relapses. Women who are either pregnant or in the postpartum period make up 10-25% of TTP patients, suggesting the interrelationship between TTP and pregnancy. The introduction of aggressive treatment with plasma transfusion and plasmapheresis has improved maternal and fetal survival rates. We report four cases of pregnancy-related TTP, describing the clinical course of patients, including response to therapy and pregnancy outcomes. Three out of four (75%) patients were treated by daily single session of plasmapheresis for a period ranged between 3 and 23 days. One patient had complete response to treatment with no sequelae, the second patient had resistant disease and died due to multiorgan failure, while the third patient had complete response after 2 episodes of TTP, which was complicated by intrauterine fetal growth retardation and death. Review of the previously reported cases of pregnancy-related TTP and the current treatment options for this rare condition are discussed also.     

Thrombotic thrombocytopenic purpura: a hematological emergency

Background

Thrombotic thrombocytopenic purpura is a hematological emergency and diagnostic challenge. The critical determinant of outcome is timely diagnosis and treatment.

Objectives

Describe the pathophysiology, presentation, diagnosis, and treatment of thrombotic thrombocytopenic purpura.

Discussion

Thrombotic thrombocytopenic purpura has a varied presentation and a tendency to mimic several disorders. However, it may be at least provisionally diagnosed in the patient with thrombocytopenia and microangiopathic hemolytic anemia without alternate cause. The mainstay of treatment is immediate plasma exchange to be repeated until platelet count is stabilized. Adjuvant therapies include corticosteroids, rituximab, and cyclosporine.

Conclusion

It is essential for the emergency physician to be aware of thrombotic thrombocytopenic purpura’s range of presentations, diagnostic criteria, and treatment.     

Thrombotic thrombocytopenic purpura (TTP) presenting as pancreatitis

Thrombotic thrombocytopenic purpura (TTP) is a rare clinical entity. It is a multi-systemic disorder characterized by a clinical pentad of thrombocytopenia, microangiopathic hemolytic anemia, diffuse and nonfocal neurologic symptoms, decreased renal function, and fever. Abdominal pain is an uncommon presenting symptom for TTP. Pancreatitis may occur from TTP or, in a few cases, may trigger TTP. The clinical diagnosis of TTP is generally difficult because there are many varied clinical presentations and the full expression of the pentad may be prolonged. However, once the diagnosis is suspected or confirmed, immediate plasmapherseis with plasma exchange must be performed to reduce the severe morbidity from neurologic disability.     

Thrombotic thrombocytopenic purpura

This overview summarizes the history of thrombotic thrombocytopenic purpura (TTP) from its initial recognition in 1924 as a most often fatal disease to the discovery in 1997 of ADAMTS-13 deficiency as a major risk factor for acute disease manifestation. The cloning of the metalloprotease, ADAMTS-13, an essential regulator of the extremely adhesive unusually large von Willebrand factor (VWF) multimers secreted by endothelial cells, as well as ADAMTS-13 structure and function are reviewed. The complex, initially devised assays for ADAMTS-13 activity and the possible limitations of static in vitro assays are described. A new, simple assay using a recombinant 73-amino acid VWF peptide as substrate will hopefully be useful. Hereditary TTP caused by homozygous or double heterozygous ADAMTS-13 mutations and the nature of the mutations so far identified are discussed. Recognition of this condition by clinicians is of utmost importance, because it can be easily treated and--if untreated--frequently results in death. Acquired TTP is often but not always associated with severe, autoantibody-mediated ADAMTS-13 deficiency. The pathogenesis of cases without severe deficiency of the VWF-cleaving protease remains unknown, affected patients cannot be distinguished clinically from those with severely decreased ADAMTS-13 activity. Survivors of acute TTP, especially those with autoantibody-induced ADAMTS-13 deficiency, are at a high risk for relapse, as are patients with hereditary TTP. Patients with thrombotic microangiopathies (TMA) associated with hematopoietic stem cell transplantation, neo-plasia and several drugs, usually have normal or only moderately reduced ADAMTS-13 activity, with the exception of ticlopidine-induced TMA. Diarrhea-positive-hemolytic uremic syndrome (D+ HUS), mainly occurring in children is due to enterohemorrhagic Escherichia coli infection, and cases with atypical, D- HUS may be associated with factor H abnormalities. Treatment of acquired idiopathic TTP involves plasma exchange with fresh frozen plasma (FFP), and probably immunosuppression with corticosteroids is indicated. We believe that, at present, patients without severe acquired ADAMTS-13 deficiency should be treated with plasma exchange as well, until better strategies become available. Constitutional TTP can be treated by simple FFP infusion that rapidly reverses acute disease and--given prophylactically every 2-3 weeks--prevents relapses. There remains a large research agenda to improve diagnosis of TMA, gain further insight into the pathophysiology of the various TMA and to improve and possibly tailor the management of affected patients.     

Thrombotic thrombocytopenic purpura associated with pesticides: A report of 4 cases and literature review

Thrombotic thrombocytopenic purpura (TTP) is a disease characterized by the presence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, caused by the congenital or acquired decrease of the enzyme activity which degrades unusual large vWF multimers. There is no identifiable cause in half of the acquired TTP cases. Herein, we report four possible pesticide-related cases with decreased ADAMTS13 enzyme activity, increased titer of ADAMTS13 inhibitor and typical clinical and laboratory presentation.     

血栓性血小板减少性紫癜误诊1例

患者,女,19岁,因“发热、腹泻3d,伴牙龈出血、右肩右髋部出血点1d”,至我院急诊就诊。患者入院前3d无明显诱因出现腹泻、呕吐、高热、腹痛,腹泻初为水样便,量多,后逐渐为黑色便,发热体温最高达40℃。在家自服去痛片、阿莫西林、安乃近等药。入院前1d出现牙龈出血,伴右肩、右髋部出血点。来诊时急查化验示血常规：     

Coexistence of thrombotic thrombocytopenic purpura and immune thrombocytopenic purpura in an Asian woman: a case report

A 33-year-old Chinese woman with a history of immune thrombocytopenic purpura presented with heavy menstrual bleeding. She was found to have thrombocytopenia, plasma ADAMTS13 activity of 0%, and positivity for the plasma ADAMTS13 inhibitor. She was diagnosed with the coexistence of thrombotic thrombocytopenic purpura and immune thrombocytopenic purpura. The patient was treated by plasmapheresis, a glucocorticoid, and rituximab. Her platelet level returned to normal, and she was discharged 28 days after admission. The number of plasmapheresis sessions and the timing of rituximab administration may be the key aspects of management of patients with thrombotic thrombocytopenic purpura who have underlying immune dysfunction caused by diseases such as immune thrombocytopenic purpura.     

Thrombotic thrombocytopenic purpura: Evolution across 15 years

Thrombotic thrombocytopenic purpura (TTP) was originally described 70 years ago. It is considered an uncommon disorder with a reported occurrence rate of one case per 1 million patients. Mortality has decreased from almost 100% early on to 30–50% with the advent of newer treatment methods. We reviewed 41 patients with a diagnosis of TTP spanning the years 1980 to mid 1994. We found a much higher case rate, one per 6000 hosptial admissions, and an overall death rate of 40%. However, isolating 5 year periods we noted a marked fall in mortality from 54% (1980–1984), 44% (1985–1989), to 18% (1990–1994). Previous reports describe relapsing TTP and report an incidence of 7–15% although very recent data suggests a higher incidence. In our study, we found an overall relapse rate of 25% and by 5 year periods 23% (1980–1984), 13% (1985–1989), and 46% (1990–1994). We suggest that the improvement in survival and the increase in relapse rate are related and reflect more effective therapy for this once almost always fatal disease. Patients now survive their initial episode and thus are at risk for recurrence. Identification of risk factors for relapse will require further study. © 1996 Wiley-Liss, Inc.     

Thrombotic thrombocytopenic purpura mimicking acute ischemic stroke

Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by thrombocytopenia, haemolytic anemia, fluctuating neurological deficits, fever, and renal impairment. This case report is about a young man who presented with acute onset right sided paralysis, dysarthria, and central facial paralysis, suggestive of cerebrovascular accident, but eventually diagnosed as TTP. In addition, the clinical presentation of TTP is discussed and some teaching points for the emergency physicians are emphasised.