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。     

妊娠合并血小板减少108例临床分析

目的探讨妊娠合并血小板减少的原因及围生期处理。方法1995-2005对南通大学附属医院收治的108例妊娠合并血小板减少患者的临床资料进行回顾性分析。结果妊娠合并血小板减少的主要原因有妊娠相关性血小板减少(PAT),特发性血小板减少性紫癜(ITP)和妊娠期高血压疾病。PAT一般不需特殊处理。血小板<50×109/L,或有明显出血倾向时,给予糖皮质激素或(和)免疫球蛋白治疗;输注血小板只用于血小板<50×109/L,且有明显出血倾向,或紧急手术前。分娩方式视血小板多少及有无产科指征而定。结论妊娠合并血小板减少应视不同的病因、血小板减少的轻重程度以及病情的缓急状况,而采取不同的处理方式。     

产后特发性急性肾功能衰竭

产后特发性急性肾功能衰竭的临床特点为产褥期发生的微血管病性溶血性贫血、血小板减少症及肾功能衰竭三联征。因其病因未明,治疗尚属推理性。肾脏为主要受累器官,其病理改变与临床预后密切相关。对重症病例的治疗主张应用抗血小板聚集剂前列腺环素(PGI_2)和抗凝血酶Ⅲ(AT-Ⅲ),输入新鲜冷冻血浆和血浆交换,以求改善患者的预后。     

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。     

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。     

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.     

妊娠合并血小板减少112例临床分析

目的:探讨妊娠合并血小板减少的发病机制及围生期的处理方法。方法:回顾分析天津医科大学总医院1992年至2002年间112例妊娠合并血小板减少患者的病因及临床处理经验。结果:112例孕妇中特发性血小板减少性紫癜27例,占24.1%;子痫前期44例,占39.3%;妊娠期特发性血小板减少40例,占34.8%;血栓性血小板减少性紫癜1例,占0.89%。阴道分娩27例,剖宫产85例。产后出血13例,产褥感染1例。结论:多种原因可以导致妊娠妇女血小板减少。如无产科指征,以阴道分娩为宜;血小板<50×109/L时,在血源充足时行剖宫产。不主张采用侵入性检查方法确定胎儿血小板水平。     

113例妊娠合并血小板减少临床分析

目的:讨论妊娠合并血小板减少的原因,处理及预后。方法:回顾性分析我院2009年～2011年收治的113例妊娠合并血小板减少患者的临床资料。结果:妊娠合并血小板减少的原因最常见的是妊娠相关性血小板减少(PAT),其次为妊娠期高血压疾病及免疫性血小板减少性(ITP)。分娩方式由产科指征决定。113例患者均预后良好。结论:对于妊娠合并血小板减少应明确病因,针对病因、血小板减少的程度及有无症状,采取不同的处理方法。血小板减少绝大多数均可在较短时间内恢复正常,结局较好。     

妊娠合并血小板减少208例临床分析

目的:探讨妊娠合并血小板减少的病因及围生期的处理方法。方法:回顾性分析1996年1月至2005年12月收治的妊娠合并血小板减少患者208例临床资料。结果:208例孕妇中妊娠期血小板减少症(PAT)88例(42.31%),特发性血小板减少性紫癜(ITP)58例(27.88%),妊娠期高血压疾病32例(15.38%),系统性红斑狼疮12例(5.77%),再生障碍性贫血(AA)10例(4.81%),妊娠期肝内胆汁淤积症(ICP)2例(0.96%),血栓性血小板减少性紫癜(TTP)2例(0.96%),Evan’s综合征1例(0.48%),病因不明3例(1.44%)。结论:多种原因可引起妊娠期孕妇血小板减少,PAT是最常见类型。血小板<50×109/L,应在术前输注浓缩血小板后行剖宫产;血小板计数>50×109/L的孕妇,如无产科指征,应阴道分娩为主。     

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。     

Successful pregnancies of two patients with relapsing thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura is a severe multisystemic disorder of unknown origin. The association of relapsing TTP with pregnancy is rare but well documented and high mortality rates of mothers and fetuses have been reported so far. Since the introduction of plasma therapy for treating the acute exacerbations of the disease, overall mortality rates have decreased significantly. It is now evident that the manifestations of the disease may reappear even after long disease-free intervals and as many as a third of the recovering patients may develop a relapse. Presented are two TTP patients with relapsing TTP complicating their pregnancies. Prophylactic treatment with aspirin and dipyridamole during their last three successful pregnancies prevented or minimized the severity of TTP relapses. The course of these pregnancies and the management of such patients is discussed.     

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.     

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.     

Treatment of postpartum thrombotic microangiopathy with plasma exchange using cryosupernatant as replacement

Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are rare but acknowledged problems of pregnancy and the postpartum period. These diseases together with thrombotic angiopathy are associated with high maternal and fetal mortality and severe long-term morbidity. We describe four women with postpartum HUS/TTP treated with plasma exchange cryosupernatant fraction of plasma (CFP) as replacement fluid. Anuria or oliguria at the beginning of treatment and thrombocytopenia [thromb- (29-68) x 109/L] were common features. Two of the patients developed a prolonged and more difficult clinical condition affecting the central nervous system and the liver and their platelet counts remained low despite the plasma exchange. The renal and hepatic function of all of the patients recovered fully. This small analysis lends weight to early plasma exchange with cryosupernatant as part of the treatment of postpartum HUS/TTP and suggests that persistent thrombocytopenia is a signal of more serious disease.     

Thrombotic thrombocytopenic purpura: medical and biological monitoring of six pregnancies

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare cause of severe thrombocytopenia in pregnancy. METHODS: Six pregnancies in five patients with TTP were followed prospectively over 5 years. Ultralarge von Willebrand factor (ULvWF) multimers and cleaving protease (cp) levels were measured. RESULTS: TTP relapsed, complicating four of the six pregnancies. Of three patients who relapsed, two had complete or partial vWF-cleaving protease (vWF-cp) deficiency, and one had a normal vWF-cleaving protease level. In all three we found abnormal UL multimers. The two women who did not relapse had normal vWF-cleaving protease level and an absence or loss of UL multimers. CONCLUSIONS: Pregnant patients with a history of TTP must be followed in a tertiary obstetric unit with plasmapheresis available. Influence of vWF-cleaving protease and vWF multimeric abnormalities on TTP relapsing during pregnancy has to be evaluated in a further multicentre study.     

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.     

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.     

Púrpura trombótica trombocitopénica versus síndrome de HELLP: un reto diagnóstico durante la gestación

Thrombocytopenia is relatively frequent during pregnancy and can occur in a range of syndromes. Among these, particular attention should be paid to thrombotic thrombocytopenic purpura (TTP) and HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count). It can be difficult to differentiate between these conditions during pregnancy, and their management and complications may differ widely. It is therefore essential to recognize their causes and perform a correct differential diagnosis, particularly in light of their possible serious consequences, which include maternal and/or fetal death.     

叶酸联合甲古胺对妊娠期高血压疾病患者高同型半胱氨酸血症的影响

目的　研究血浆总同型半胱氨酸 (tHcy)水平与妊娠期高血压疾病的关系及叶酸联合甲古胺对其的影响。方法　 2 0 0 1年 1月至 2 0 0 3年 6月广东省东莞市人民医院采用荧光偏振免疫分析法测定 16 2例妊娠期高血压疾病患者和 15 0例正常晚期妊娠妇女血浆tHcy水平 ,同时采用离子捕捉免疫分析法检测其血清叶酸水平和微粒子酶联免疫分析法检测其血清VitB1 2 水平 ,并对 36例合并高Hcy血症的患者采用叶酸联合甲古胺进行干预治疗 ,4周后再次测定其血浆tHcy水平、血清叶酸和VitB1 2 水平。结果　妊娠期高血压疾病患者血浆tHcy水平显著高于正常妊娠组 (P <0 0 1) ,血清叶酸和VitB1 2 水平显著低于正常妊娠组 (P <0 .0 5 ) ;36例合并高Hcy血症的患者治疗后血浆tHcy水平比治疗前显著下降 (P <0 0 1) ,而血清叶酸和VitB1 2 水平比治疗前则显著升高 (P <0 0 5 )。结论　高Hcy血症是妊娠期高血压疾病发病的重要因素之一 ,应用叶酸联合甲古胺治疗 ,可显著降低妊娠期高血压疾病合并高Hcy血症患者的血浆tHcy水平 ,减少高Hcy对血管的毒性作用 ,有助于改善妊娠期高血压疾病患者的疾病转归。     

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.