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.     

产后并发血栓性血小板减少性紫癜一例分析

血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)是临床罕见的急性严重性疾病,发病率为3.7/百万人,死亡率高达50％-80％,病因不明。目前认为,可能与感染、药物、免疫、妊娠、肿瘤等因素有关。孕产妇发生TTP更为罕见。我院产科收治1例产后并发TTP的患者,现结合文献复习报道如下。     

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。     

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.     

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     

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。     

妊娠合并急性胆石症及血栓性血小板减少性紫癜1例

本文报道1例22岁孕妇, 孕3产0, 贫血、血小板减低2年, 因"宫内孕19+3周, 头痛1个月, 腹痛3 d, 皮肤黄染1 d"入院。查体皮肤黄染、瘀斑, 口唇苍白, 肝肾功能受损、胆红素显著升高;影像学检查:肝内外胆管扩张。初步诊断:宫内中孕期, 胎死宫内, 梗阻性黄疸, 血小板减少, 中度贫血。经多学科会诊, 急诊行支架引流及对症治疗, 黄疸缓解;外周血细胞形态分析见破碎红细胞3%, 于血浆置换同时行药物引产顺利。血管性血友病因子裂解蛋白酶(ADAMTS13)活性<1%, ADAMTS13抑制物阳性, 诊断为获得性血栓性血小板减少性紫癜(TTP), 予血浆置换、糖皮质激素及利妥昔单抗治疗, 病情缓解出院。妊娠合并急性胆石症及TTP极罕见, 危及母胎安全, 本文结合文献及诊疗经过, 分析其诊断及干预措施。     

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。     

妊娠合并血小板减少诊断及处理

妊娠合并血小板减少是指在妊娠期由不同的病因引起的血小板低于正常范围,其中妊娠相关性血小板减少症(PAT)、妊娠合并特发性血小板减少性紫癜(ITP)和妊娠期高血压疾病是其主要病因。妊娠合并血小板减少的治疗关键在于找出病因,进行严密监测,严重的血小板减少症需要接受一系列的治疗,包括糖皮质激素、免疫球蛋白的输注等,但血小板的输注要十分慎重,对于血栓性血小板减少性紫癜(TTP)的病人输注血小板为禁忌。     

经阴道超声与超声造影应用于早孕期瘢痕妊娠中的诊断价值分析

目的 分析早孕期瘢痕妊娠应用经阴道超声与超声造影诊断的价值。方法 选取42例疑似早孕期瘢痕妊娠患者,患者均实施经阴道超声与超声造影检查,比较经阴道超声与超声造影不同检查方法瘢痕诊断、病理结果及诊断效能。结果 经术后病理结果证实,瘢痕妊娠患者31例,非瘢痕妊娠患者11例;经阴道超声诊断：瘢痕妊娠29例,非瘢痕妊娠13例;超声造影诊断：瘢痕妊娠30例,非瘢痕妊娠12例。超声造影检查准确率、特异度及敏感度均高于经阴道超声检查（P<0.05）。结论 超声造影与经阴道超声应用于早孕期瘢痕妊娠诊断中,超声造影检查的结果更接近于病理结果,且获取的超声图像较经阴道超声更为清晰,能清晰观察明确妊娠物着床位置,并体现其与子宫肌层的关系,相比经阴道超声检查具有更高的瘢痕妊娠诊断准确率、特异度和敏感度,有利于疾病的分型诊断,具有较高的应用价值。     

The obfuscation of eclampsia by thrombotic thrombocytopenic purpura

All case reports of TTP in pregnancy were reviewed. In some cases the primary diagnosis of TTP may have been inappropriate, with severe pre-eclampsia or eclampsia being the primary problem. Some cases of eclampsia and severe pre-eclampsia satisfy all the criteria for the diagnosis of TTP syndrome. However, the prognosis is much better and management of these patients is very different from the nonpregnant patient with the TTP syndrome. The use of the term TTP syndrome to describe these patients may be confusing. Two cases of eclampsia are presented where the diagnosis of TTP could have been made but would have been inappropriate.     

A high LDH to AST ratio helps to differentiate pregnancy-associated thrombotic thrombocytopenic purpura (TTP) from HELLP syndrome

Objective: Differentiating between pre-eclampsia/HELLP syndrome and pregnancy-associated thrombotic thrombocytopenic purpura (TTP) is difficult but important in order to undertake timely and potentially life-saving plasma exchange (PEX) therapy for TTP recovery. We review our institutional experience with pregnancy-associated TTP and determine if the ratio of LDH to AST reliably distinguishes patients with TTP from those with HELLP syndrome. Study design: This is a retrospective case control study of all pregnant/puerperal patients with TTP from a single tertiary care center during 1986–2006. Laboratory findings in patients with TTP were compared to patients who met all criteria for class 1 or 2 HELLP syndrome within the first 24 hours of hospital admission during 2000–2007. Results: Thirteen pregnant (n?=?10) or puerperal (n?=?3) patients with TTP were identified; 11 cases were primary, 2 were recurrent. TTP laboratory findings included LDH to AST ratios of 77?±?42.17; Patients with HELLP syndrome (N?=?83) had significantly lower LDH to AST ratios of 20.04?±?2.13. Based on an ROC analysis, an LDH/AST ratio ≥22.12 discriminates well between TTP and antenatal HELLP subjects (AUC?=?0.99). Conclusion: A high LDH to AST ratio >22.12 suggests that TTP is a more likely diagnosis than HELLP syndrome in the third trimester pregnant patient, presenting with findings that could be compatible with either diagnosis. In these circumstances, it is advisable to obtain hematology consultation and to consider PEX implementation.     

Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006

A review of pregnancy-associated thrombotic thrombocytopenic purpura (TTP) in 166 pregnancies was undertaken using 92 English-language publications from 1955 to 2006. Initial and recurrent TTP presents most often in the second trimester (55.5%) after 1-2 days of signs/symptoms; postpartum TTP usually occurs following term delivery. TTP with preeclampsia (n = 28) exhibits 2-4 times higher aspartate aminotransferase (AST) values and lower total lactate dehydrogenase (LDH) to AST ratios (LDH to AST ratio = 13:1), compared with TTP without preeclampsia (LDH to AST ratio = 29:1). Maternal mortality is higher with initial TTP (26% vs 10.7%), especially with concurrent preeclampsia (44.4% vs 21.8%, P < .02). Although maternal mortality with TTP has substantially declined when plasma therapy is utilized, delay of diagnosis and therapy for initial TTP confounded by preeclampsia/hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome remains a significant maternal-perinatal threat. Rapid and readily available laboratory testing to quickly diagnose TTP and HELLP syndrome/preeclampsia is desperately needed to improve care.     

Congenital thrombotic thrombocytopenic purpura: Upshaw–Schulman syndrome: a cause of neonatal death and review of literature

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in children characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. The classic Moschcowitz Pentads of TTP include hemolytic anemia, with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decrease renal function and fever. We report a newborn who was diagnosed with congenital TTP. The newborn was admitted at age of 40 h, in our hospital, in view of respiratory distress with impending respiratory failure and red colored urine. On examination, the newborn was febrile, tachypneic, had deep icterus, pallor and no hepatosplenomegaly. Family history was significant with one unexplained neonatal death at age of 24 with symptoms of red colored urine. Examination of peripheral smear was diagnostic with the presence of fragmented RBCS, giant but fewer platelets consistent with a diagnosis of MAHA. The diagnosis of TTP was confirmed with low ADAMTS activity and gene analysis showed c 2203 G > T-p.Glu735X (domain TSP1-2) mutation in exon 18 of ADAMTS 13 gene. The newborn had rapid deterioration, with respiratory distress and refractory shock leading to death. Post-mortem bone marrow done showed marrow hyperplasia.     

Thrombotic thrombocytopenic purpura in pregnancy

Summary. A 20 week primigravida with intrauterine fetal death and the rare occurrence of thrombotic thrombocytopenic purpura (TTP) in pregnancy is presented. Aprotinin has been used to overcome uterine inertia non-responsive to oxytocin stimulation. TTP was successfully treated with repeated plasmaphereses. The literature is briefly reviewed and the complexity and importance of the differential diagnosis specific to the pregnant patient is discussed.     

Purpura thrombotique thrombocytopénique au cours de la grossesse : une entité rare à ne pas méconnaître !

We report a case of pregnancy-associated thrombotic thrombocytopenic purpura (TTP) coupled with severe pre-eclampsia at 30 weeks of gestation. TTP, qualified also as imitator of HELLP syndrome because of similar clinical and biological presentations, is a difficult diagnosis. It must be considered in any severe thrombocytopenia, coupled with minimal cytolysis or absent. Treatment based on plasma exchanges must be initiated as soon as possible without waiting for laboratory confirmation of the diagnosis, the determination of plasma ADAMTS 13 activity requiring several days. Except of case of life-threatening bleeding, any platelet transfusion should be prohibited as it would increase microvascular thrombosis.     

Thrombotic thrombocytopenic purpura--a rare and difficult differential diagnosis to HELLP syndrome in late pregnancy

Thrombotic thrombocytopenic purpura (TTP) represents a rare complication mainly in the second trimester and can, in its early stage, mimic HELLP syndrome. At 38 weeks of gestation, a 40-year-old primigravida with elevated blood pressure, thrombocytopenia and elevated liver enzymes was diagnosed with HELLP syndrome. Despite a Ceasarean section her laboratory parameters remained pathological with severe thrombocytopenia and hemolysis. In addition, the patient developed neurological comatose-like symptoms. The diagnosis of TTP was made after further tests revealed fragmented red cells in the blood, autoantibodies against ADAMTS13 and a nearly total loss of ADAMST13 plasma activity. The patient fully recovered following repeated plasmapheresis and plasma substitution as well as systemic treatment with dexamethasone. If laboratory parameters do not normalise promptly in spite of the correct treatment for an assumed HELLP syndrome, TTP may be the underlying pathology. As rapid assays for antibodies against ADAMTS13 are not available yet, special consideration must be given to the clinical details in order to make a correct diagnosis.     

Successful management of pregnancy-associated thrombotic thrombocytopenic purpura by monitoring ADAMTS13 activity

Thrombotic thrombocytopenic purpura (TTP) during pregnancy is very rare and is caused by an absent or severely depleted ADAMTS13 (a disintegrin-like and metallopeptidase with thrombospondin type 1 motif, 13). A 37-year-old multigravida woman developed TTP with severe anemia and thrombocytopenia at 22 weeks' gestation. ADAMTS13 activity was markedly decreased to 3% and ADAMTS13 inhibitor was positive, leading to a definitive diagnosis of TTP. She was successfully treated by plasmapheresis six times, resulting in symptomatic relief. Close follow up with periodic ADAMTS13 measurement facilitated plasmapheresis at appropriate points at a minimum frequency during pregnancy. Because of intrauterine growth retardation from 28 weeks' gestation, an elective cesarean section was performed at 30 weeks' gestation. After delivery, the mother and child showed no appreciable problem. To our knowledge, this is the first report of successful management for pregnancy-associated TTP by monitoring ADAMTS13 activity during pregnancy and the postpartum period.     

Bilateral serous retinal detachment as a complication of acquired peripartum thrombotic thrombocytopenic purpura bout

We report a case of a 26-year-old primigravid woman, believed to have HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome, which turned out to be a thrombotic thrombocytopenic purpura (TTP) bout. At the 40th gestational week, based on the clinical picture of HELLP syndrome, a cesarean section was performed and a dysmature male newborn was delivered. Afterwards, clinical symptoms and laboratory abnormalities persisted. Severe ADAMTS13 deficiency with the presence of inhibitory anti-ADAMTS13 antibodies revealed acquired thrombotic thrombocytopenic purpura bout, which was complicated with bilateral vision decrease due to bilateral retinal detachment. At the first ophthalmological examination, ultrasonography and binocular indirect ophthalmoscopy confirmed the diagnosis of the serous retinal detachment. After the diagnosis of acquired TTP bout, the patient was treated with multiple plasmapheresis and intravenous immunoglobulin with rapid improvement of the clinical and laboratory parameters. The ophthalmologic complications disappeared later without sequelae. At the 18-month examination, substantial visual acuity improvement without serous retinal detachment and full best corrected visual acuity were observed.     

Thrombotic thrombocytopenic purpura in pregnancy. A case report

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. About 10-20% of TTP cases are associated with the pregnancy. Preterm delivery and intrauterine fetal death are frequent pregnancy complications of TTP. The following paper presents the case of a 32-year-old woman with TTP relapse at 10 weeks of her second pregnancy. Despite regular fresh frozen plasma transfusions, intrauterine fetal death occurred at 21 weeks of gestation. Current views on TTP management during pregnancy have been presented in the article as well.