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妊娠合并血栓性血小板减少性紫癜和获得性免疫缺陷综合征:病例报告。

Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report.

机构信息

Department of Obstetrics and Gynecology of Hospital de Clínicas de Porto Alegre, Rio Grande do Sul, Brazil.

出版信息

J Obstet Gynaecol Res. 2021 May;47(5):1898-1902. doi: 10.1111/jog.14717. Epub 2021 Mar 14.

DOI:10.1111/jog.14717
PMID:33719122
Abstract

Thrombotic thrombocytopenic purpura (TTP) is a medical emergency that demands prompt diagnosis to allow lifesaving treatment: plasmapheresis. TTP during pregnancy is rare, with estimated prevalence of 1/200 000, and even rarer in association with acquired immunodeficiency syndrome, with only two cases reported. Differential diagnosis includes HELLP syndrome (hemolysis elevated liver enzymes and low platelets), sepsis, intravascular-disseminated coagulation, and acquired autoimmune diseases, each one with its unique treatment and prognosis. A case of a pregnant woman at 26th week with sudden onset of left hand paresthesia and purpura is reported. PLASMIC score showed high risk for ADAMTS-13 deficiency and diagnosis of TTP was made. Human immunodeficiency virus screening test was positive on admission. Plasmapheresis and antiretroviral therapy were initiated and the delivery of a healthy newborn at full-term gestation was achieved, unlike other cases in literature. No obstetric complications were observed and the follow-up shows no signs of disease recurrence.

摘要

血栓性血小板减少性紫癜(TTP)是一种医学急症,需要及时诊断以进行救生治疗:血浆置换。妊娠期间 TTP 罕见,估计患病率为 1/200000,与获得性免疫缺陷综合征相关的 TTP 更为罕见,仅有两例报告。鉴别诊断包括 HELLP 综合征(溶血、肝酶升高和血小板减少)、脓毒症、血管内弥散性凝血和获得性自身免疫性疾病,每种疾病都有其独特的治疗和预后。报告了一例 26 周孕妇突发左手麻木和紫癜的病例。PLASMIC 评分显示 ADAMTS-13 缺乏的高风险,诊断为 TTP。入院时人类免疫缺陷病毒筛查试验呈阳性。开始进行血浆置换和抗逆转录病毒治疗,并足月分娩了一名健康的新生儿,与文献中的其他病例不同。未观察到产科并发症,随访未发现疾病复发迹象。

相似文献

1
Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report.妊娠合并血栓性血小板减少性紫癜和获得性免疫缺陷综合征:病例报告。
J Obstet Gynaecol Res. 2021 May;47(5):1898-1902. doi: 10.1111/jog.14717. Epub 2021 Mar 14.
2
Severe HELLP syndrome masquerading as thrombocytopenic thrombotic purpura: a case report.严重的 HELLP 综合征伪装为血小板减少性血栓性紫癜:一例报告。
BMC Nephrol. 2020 May 29;21(1):204. doi: 10.1186/s12882-020-01865-y.
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Postpartum plasma exchange in a woman with suspected thrombotic thrombocytopenic purpura (TTP) vs. hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP): a case study.疑似血栓性血小板减少性紫癜(TTP)与溶血、肝酶升高及血小板减少综合征(HELLP)的产后血浆置换:病例研究
Nephrol Nurs J. 2010 Jul-Aug;37(4):399-402.
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Thrombotic thrombocytopenic purpura masquerading as hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in late pregnancy.妊娠晚期表现为溶血、肝酶升高、血小板减少(HELLP)综合征的血栓性血小板减少性紫癜。
Obstet Gynecol. 2006 Sep;108(3 Pt 2):817-20. doi: 10.1097/01.AOG.0000215994.25958.31.
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Thrombotic thrombocytopenic purpura in a patient with acquired immunodeficiency syndrome at 28 weeks gestation.一名妊娠28周的获得性免疫缺陷综合征患者发生血栓性血小板减少性紫癜。
Am J Perinatol. 2005 May;22(4):223-5. doi: 10.1055/s-2005-866605.
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Incidence of obstetrical thrombotic thrombocytopenic purpura in a retrospective study within thrombocytopenic pregnant women. A difficult diagnosis and a treatable disease.一项针对血小板减少孕妇的回顾性研究中产科血栓性血小板减少性紫癜的发病率。一种诊断困难但可治疗的疾病。
BMC Pregnancy Childbirth. 2015 Jun 17;15:137. doi: 10.1186/s12884-015-0557-5.
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Differentiation between severe HELLP syndrome and thrombotic microangiopathy, thrombotic thrombocytopenic purpura and other imitators.重度HELLP综合征与血栓性微血管病、血栓性血小板减少性紫癜及其他模仿者之间的鉴别。
Eur J Obstet Gynecol Reprod Biol. 2015 Jun;189:68-72. doi: 10.1016/j.ejogrb.2015.03.017. Epub 2015 Mar 25.
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Postpartum thrombotic thrombocytopenic purpura complicated by Budd-Chiari syndrome.产后血栓性血小板减少性紫癜合并布加综合征
Obstet Gynecol. 1995 May;85(5 Pt 2):839-43. doi: 10.1016/0029-7844(94)00407-5.
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[Thrombotic thrombocytopenic purpura (TTP)--a difficult differential diagnosis to other microangiopathies in pregnancy].[血栓性血小板减少性紫癜(TTP)——妊娠中与其他微血管病的鉴别诊断难点]
MMW Fortschr Med. 2011 Jan 27;153(4):48-9. doi: 10.1007/BF03367701.
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Bilateral serous retinal detachment as a complication of acquired peripartum thrombotic thrombocytopenic purpura bout.双侧浆液性视网膜脱离作为获得性围产期血栓性血小板减少性紫癜发作的一种并发症。
J Obstet Gynaecol Res. 2011 Oct;37(10):1506-9. doi: 10.1111/j.1447-0756.2011.01545.x. Epub 2011 May 22.

引用本文的文献

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A Race against the Clock: A Case Report and Literature Review Concerning the Importance of ADAMTS13 Testing in Diagnosis and Management of Thrombotic Thrombocytopenic Purpura during Pregnancy.与时间赛跑:关于ADAMTS13检测在妊娠期血栓性血小板减少性紫癜诊断和管理中的重要性的病例报告及文献综述
Diagnostics (Basel). 2022 Jun 27;12(7):1559. doi: 10.3390/diagnostics12071559.