妊娠期血栓性血小板减少性紫癜和溶血性尿毒症综合征的识别与治疗

血栓性血小板减少性紫癜（TTP）与溶血性尿毒症综合征（HUS）均属于血栓性微血管病,起病急、病情重、病死率高。虽然不是妊娠期特有疾病,但是因临床稀发,并且与常见的妊娠期特有疾病（重度子痫前期、HELLP综合征）临床表现具有显著同质化,容易被漏诊误诊。文章就其临床表现、发病机制、诊断标准、鉴别诊断、治疗方案进行总结归纳,旨在为临床医生判断和救治提供借鉴。     

妊娠期血栓性血小板减少性紫癜的发病机制与诊治

血栓性血小板减少性紫癜(thrombotic thrombocy-topenia purpura，TTP)为一罕见的微血管血栓性综合征，其主要特征为发热、血小板减少性紫癜、微血管溶血性贫血、中枢神经系统和肾脏受累等，成为五联症。本病的病因目前尚不清楚。当与妊娠合并存在时严重威胁母婴生命。     

4例妊娠合并血栓性微血管病的临床观察

目的:通过4例妊娠合并血栓性微血管病[TMA,其中溶血性尿毒症综合征(HUS)和血栓性血小板减少性紫癜(TTP)各2例]患者的临床资料分析,探讨其诊断与治疗.方法:通过病史记录,对临床、实验室检查和(或)病理诊断的4例TMA资料进行回顾性分析.结果:4例患者中3例初产妇,1例经产妇.3例患者孕龄32～37周,均死胎,母亲均存活;1例患者24周并发自然流产,入院后死亡.剖宫产1例,阴道分娩2例.实验室检查:4例患者Hb 29.0～57.0 g/L,Plt14×109/L～40×109/L,外周血片中均见破碎红细胞;4例患者均有肾功能损害,2例患者分娩后即无尿,其中1例患者行肾脏穿刺,病理报告符合HUS.存活的3例患者都接受血浆、洗涤红细胞、血小板治疗和(或)持续肾替代治疗(CRRT).2例HUS患者行血液透析治疗,1例患者行,TTP血浆置换.结论:妊娠合并TTP、HUS的母儿死亡率高,内皮细胞损伤可能是继发导致微血管血栓形成的一个重要原因,产科医生必须对此病提高警惕,早期诊断、早期输血浆或血浆置换,能提高母儿生存率.     

妊娠合并血栓性血小板减少性紫癜的研究进展

血栓性血小板减少性紫癜（TTP）是罕见的血液科致死性疾病,围生期更为罕见。临床常表现为三联征（微血管性溶血性贫血、进行性血小板减少、神经系统异常）或五联征（三联征加发热及肾脏损害）。TTP发病机制不清,可能为遗传性血管性血友病因子裂解酶缺失,或妊娠期血管性血友病因子裂解酶分泌增加所致。妊娠为其发作的独立诱因,母儿预后不良,围生期规范使用血浆置换、血浆输注等综合治疗,适时终止妊娠,可改善预后。     

HELLP综合征和HUS-TTP的诊断和鉴别诊断

<正>HELLP综合征与溶血性尿毒症综合征(hemolytic uremia syndrome,HUS)-血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)均是妊娠严重并发症,其发病急骤,严重威胁孕产妇及胎儿生命。临床上二者因其相似的发病机制及临床症状,易引起临床医师混淆,给临床诊断、治疗带来困难。因此,充分认识HELLP综合征和HUS-TTP的诊断和鉴别诊断,对疾病的临床处理,降低患者死亡率和改善母儿预后具有重大意义。     

产后出血性尿毒症的血浆置换治疗

产后出血性尿毒症也称为溶血性尿毒综合征（ｈｅｍｏｌｙｔｉｃｕｒｅｍｉｃｓｙｎｄｒｏｍｅ，ＨＵＳ），其临床特点是微血管性溶血性贫血、急性肾功能衰竭和血小板减少。如果伴有神经系统症状和发热时，则称为血栓性血小板减少性紫癜（ｔｈｒｏｍｂｏｃｙｔｉｃｔｈｒｏ...     

一些少见的妊娠并发症

尽管既往文献曾报道过有肾功能衰竭,血清胆红素浓度上升,易出血的孕妇但未进行充分讨论,病人很可能患有严重的先兆子痫、妊娠急性脂肪肝、溶血性尿毒症综合征(HUS)或血栓性血小板减少性紫癜(TTP),这些疾病均极罕见,由于产科医师或单独处理或与消化道、血液、肾脏、神经诸科医师共同诊治,几乎无一临床医师对此综合征有许多经验或具有本专业外的诊断能力.至少有一种情况(妊娠急性脂肪肝)伴有70%的     

产后溶血性尿毒症综合征诊治进展

产后溶血性尿毒症综合征(PHUS)属于血栓性微血管疾病,是非典型溶血性尿毒症综合征中的一种,主要临床表现为产后10周内发生不可逆急性肾功能衰竭伴血小板减少、微血管病性贫血,临床罕见,预后较差,少数病例发生于妊娠晚期。目前研究认为其发病的病理生理机制为原发性或继发性补体系统调节异常。早期诊断和及时治疗可以明显改善患者预后,但其诊断与鉴别诊断较为困难,目前仍以临床诊断为主,并应尽快启动诊断性治疗,特异性基因和分子检测有助于明确诊断。血液透析、血浆置换仍然是PHUS首选的治疗方法,而针对病因的特异性补体抑制剂可用于该病的长期治疗。     

产后溶血性尿毒症性综合征21例分析

产后溶血性尿毒症性综合征２１例分析曹书华，李瑾产后溶血性尿毒症性综合征（ｐｏｓｔｐａｒｔｕｍｈｅｍｏｌｙｔｉｃｕｒｅｍｉｕｓｙｎｄｒｏｍｅ，简称产后ＨＵＳ）是一组妊娠合并急性肾功能衰竭、微血管病性溶血性贫血、血小板减少的综合征，发病率低。至今对其发病...     

妊娠合并复发型血栓性血小板减少性紫癜一例报告及文献复习

血栓性血小板减少性紫癜(TTP)属于血栓性微血管病中的一种,目前,TTP的发病机制尚不明确,临床上妊娠合并TTP也比较少见.2009年11月湖南省人民医院收治了1例妊娠合并复发型TTP患者,结合相关文献,对本病的临床特点、诊断、治疗及预后进行分析与探讨.     

产科血栓性微血管病

血栓性微血管病是一组以微血管栓塞为病理特点的综合征,起病急、病情凶险、病死率高.因孕期少见,临床医生认识不足,易漏诊误诊.文章就其分类、发病机制、临床表现、诊断标准、鉴别诊断、治疗方案及预后进行总结归纳.     

Chronic relapsing thrombotic thrombocytopenic purpura in pregnancy: A case report

Thrombotic thrombocytopenic purpura (TTP) is a disorder of unknown etiology affecting the microcirculation of multiple organ systems. Plasma therapy has significantly reduced the mortality rate; thus, an increased incidence of recurrence has been noted. Since corticosteroids, antiplatelet agents, and splenectomy do not prevent recurrences, monthly plasma infusions have been instituted to decrease the risk of recurrence. However, in pregnancy, increase in frequency of plasma infusions to weekly or biweekly intervals has been associated with avoidance of placental infarcts. This is the first report of a successful pregnancy in which bimonthly prophylactic single plasma-exchange plasmapheresis was the treatment regimen with no obvious maternal-fetal morbidity.     

Placental fetal thrombotic vasculopathy in severe congenital anomalies prompting EXIT procedure

Objective

The ex-utero intrapartum treatment (EXIT) procedure is used to secure fetal airway, cannulate for extracorporeal membrane oxygenation (ECMO), or resect a tumor during partial delivery in a modified cesarean section. This is a retrospective study of placental pathology from EXIT procedures.

Methods

Placental reports and glass slides from 36 placentas delivered by EXIT procedure (study group SG) and 36 placentas from pregnancies without perinatal mortality and delivered by cesarean sections and matched for gestational age were blindly reviewed. Indications for EXIT procedures were: 11 cervical teratomas, 9 diaphragmatic hernias, 4 pulmonary airway malformations, 4 micrognathias, 3 vascular malformations, 3 CHAOS, and 2 aortic stenoses. 22 clinical and 43 gross and histological placental features were compared using the analysis of variance or Yates χ² with Holm-Bonferroni correction, where appropriate.

Results

The average gestational age in the SG and the CG was 34.9 weeks. Histological features of fetal thrombotic vasculopathy were more frequently seen in the SG. Of the placental features, statistically significant differences were found in, partial fibrosis of chorionic villi (9.7 ± 7.9 vs. 6.1 ± 5.3 villi per placental section) [p = 0.035], clusters of at least 3 avascular chorionic villi (33 v. 6%) [p = 0.042], and abnormal umbilical cord insertion (8% vs. 0% (p = 0.045), in the SG and the CG respectively.

Conclusion

To the best of our knowledge, this is the first study to describe the placentas from EXIT procedures. The presence of increased frequency of fetal thrombotic vasculopathy on histology indicates an underlying chronic and on-going stasis in fetal circulation due to the presence of conditions which were indications for the EXIT procedures. The possibility of coagulopathy should be considered in management of the fetuses and neonates undergoing EXIT procedure. Detailed examination of the placenta is of utmost importance in order to recognize and treat potentially life-threatening complications.     

Neonatal myocardial infarction due to thrombotic occlusion

Neonatal myocardial infarction is rare and its prognosis is poor. We describe the clinical course and autopsy findings of a newborn female with myocardial infarction. Her clinical course was rapidly progressive, becoming fatal before we could detect the cause. Autopsy demonstrated significant occlusion of the left coronary artery as well as evidence of new infarction, suggesting that the event occurred at birth. This case illustrates myocardial infarction as a possible cause of early neonatal death.     

Clinical significance of placental examination in perinatal medicine

Complete pathologic evaluation of the placenta provides valuable information for perinatal care for the obstetrician, neonatologist, pediatrician, and family. The histopathology of the placenta can answer specific questions about in utero insults, give insight into management of subsequent pregnancies, and provide an assessment of the newborn risk. Placental pathology has been a key litigious informant in inferring timing of insults. Despite these well known advantages of placental pathologic examination, it remains an under-utilized part of perinatal medicine. This stems from a historically under-taught part of surgical and autopsy pathology resulting in inadequate reporting. This review will focus on the utility of the placental examination for fetal and maternal well-being. This review will be restricted to singleton births.     

妊娠合并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)可发生于任何年龄,女性多见,妊娠是诱发因素之一。本文就TTP的发病机制、妊娠与TTP的相互关系、临床表现与分类、诊断与鉴别诊断、治疗以及TTP患者妊娠前咨询等方面进行讨论。     

Adverse outcomes in obstetric-atypical haemolytic uraemic syndrome: a case series analysis

Objective: The aim of this case series is to raise awareness of obstetric-related atypical haemolytic uraemic syndrome (aHUS) amongst obstetricians and gynaecologists.

Study design: Data from 20 consecutive patients, aged 19–38, with obstetric-aHUS manifestation during or immediately after pregnancy are reported. Patients were diagnosed and treatment was initiated between 2012 and 2016.

Results: Presentation of aHUS was mainly preceded by preeclampsia and/or haemolysis, elevated liver enzymes and low platelet count syndrome, other obstetric complications, or by diarrhoea. Thrombotic microangiopathy (TMA) was evident in all patients with signs of microangiopathic haemolysis (sharp decline in haemoglobin; mean 67?g/L), elevated lactate dehydrogenase (LDH; mean 2953.1?U/L), schistocytosis, thrombocytopenia (mean platelet count 52.5?×?10⁹/µL), and acute kidney injury (AKI) (hypercreatininaemia, mean 456.4 µmol/L; oliguria or anuria). The majority of patients (80%) initially presented with arterial hypertension. Diagnosis of obstetric-aHUS was complicated, as multiple organs were affected. Time taken to make the diagnosis of aHUS delayed the initiation of fresh-frozen plasma infusions and plasma exchange (80% of patients) and subsequent eculizumab treatment (40% of patients). Maternal mortality was high (35%) as was foetal mortality (25%).

Conclusions: Obstetric-aHUS is a serious condition characterized by multiple organ failure (MOF) and a high mortality rate. Presentation of obstetric-aHUS is preceded by various precipitating factors, suggesting pregnancy complications, and not the pregnancy per se, often induce aHUS in women with a genetic predisposition to its development. A delay in the correct diagnosis and initiation of the most effective treatment can have serious consequences, reinforcing the need to raise awareness of obstetric-aHUS.