Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review

Xiaoxia CHEN; Nannan WANG; Xiaoli YAN; Dan WANG; Qing CHANG

doi:10.16016/j.2097-0927.202109074

您当前的位置：

首页 >

文章列表页 >

Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review

Clinical Medicine | 更新时间：2026-01-09

- Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review
- Journal of Army Medical University Vol. 44, Issue 4, Pages: 390-封3(2022)
- 作者机构：
  
  400038 重庆，陆军军医大学(第三军医大学)第一附属医院妇产科
- 作者简介：
- 基金信息：
- DOI：10.16016/j.2097-0927.202109074
  CLC： R554.6;R714.254
- Received：08 September 2021，
  
  Revised：2021-11-16，
  
  Published：28 February 2022
- 稿件说明：
移动端阅览
Xiaoxia CHEN, Nannan WANG, Xiaoli YAN, et al. Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review[J]. Journal of Army Medical University, 2022, 44(4): 390-封3.
DOI：

Xiaoxia CHEN, Nannan WANG, Xiaoli YAN, et al. Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review[J]. Journal of Army Medical University, 2022, 44(4): 390-封3. DOI： 10.16016/j.2097-0927.202109074.

摘要

目的

探讨早期诊治妊娠期血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura，TTP)的重要性及诊治策略。

方法

对本院2015年1月至2020年7月收治的2例妊娠合并TTP患者进行回顾性总结，分析其发病特点、诊治过程及预后，总结早期诊治的重要性及诊治策略，并对相关文献进行复习。

结果

2例患者首发症状均为血小板减少及贫血，病例1产后发病，未及时行外周血涂片明确微血管病性溶血性贫血，导致诊断延误，血浆置换延迟，患者死亡；病例2中孕期发病，及时外周血涂片发现破碎红细胞，诊断TTP，立即行血浆置换术，治愈出院。结合文献报道共纳入57例患者，分析临床资料显示TTP在妊娠期各阶段均可发病，均有不同程度微血管病性溶血性贫血和血小板减少，出现TTP三联征(微血管病溶血性贫血、血小板减少、神经系统症状)26例(45.6%)，其中死亡5例(19.2%)；合并五联征(三联征+发热、肾功能损害)7例(12.3%)，其中死亡4例(57.1%)。36例行血管性血友病因子裂解酶ADAMTS13活性检测，活性重度降低者(活性＜10%)28例(77.8%)。血浆置换组孕产妇死亡率(6.8%，3/44)明显低于未行血浆置换组(69.2%，9/13，

＜0.05)。

结论

外周血涂片是诊断微血管病性溶血的重要依据，对出现微血管病性溶血性贫血和血小板减少症状的孕产妇应高度警惕TTP可能，疑诊TTP尽快行血浆置换可明显改善母儿预后。

Abstract

Objective

To explore the importance and strategies of early diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in pregnancy.

Methods

Two cases of pregnancy complicated with TTP admitted to our hospital from January 2015 to July 2020 were recruited and retrospectively summarized. The characteristics of the disease

diagnosis and treatment process and prognosis were analyzed to summarize the importance and strategies of early diagnosis and treatment. Related literatures were reviewed.

Results

The initial symptoms were thrombocytopenia and anemia. Case 1 developed postpartum

and the diagnosis of microangiopathic hemolytic anemia was not timely confirmed by peripheral blood smear

and this resulted in delayed diagnosis

delayed plasma exchange and death of the

patient. Case 2 developed in the middle of pregnancy. Broken red blood cells were found on peripheral blood smear in time

and TTP was thus diagnosed. Plasma exchange was performed immediately

and the patient was cured and discharged. There were totally 57 patients in literature review. The results suggested that TTP can occur in all stages of pregnancy

and microangiopathic hemolytic anemia and thrombocytopenia can be the presumptive diagnosis of TTP. TTP triad (microangiopathic hemolytic anemia

thrombocytopenia

and neurological symptoms) were observed in 26 cases (45.6%)

of which 5 cases (19.2%) died; 7 cases (12.3%) were combined with pentad (triad+fever and renal dysfunction)

including 4 died (57.1%). In the 36 cases tested for the activity of von Willebrand factor lyase

ADAMTS13

28 cases (77.8%) had severely decreased activity (activity

10%). The mortality rate in the plasma exchange group (6.8%

3/44) was significantly lower than that in the group without plasma exchange (69.2%

9/13

0.05).

Conclusion

Peripheral blood smear is an important reference in the diagnosis of microangiopathic hemolysis

and the possibility of TTP should be highly vigilant for pregnant women with microangiopathic hemolytic anemia and thrombocytopenia

and plasma exchange as soon as possible after suspected TTP can significantly improve the prognosis of mothers and fetus.

关键词

Keywords

references

王夕妍, 杨仁池. 血栓性血小板减少性紫癜治疗新进展[J]. 中华血液学杂志, 2019, 40(12): 1055-1059. DOI: 10.3760/cma.j.issn.0253-2727.2019.12.020.

WANG X Y, YANG R C. Advances in the treatment of thrombotic thrombocytopenic purpura[J]. Chin J Hematol, 2019, 40(12): 1055-1059. DOI: 10.3760/cma.j.issn.0253-2727.2019.12.020.

刘燕燕, 曾万江, 徐晓燕, 等. 产后并发血栓性血小板减少性紫癜1例报告及文献复习[J]. 医学信息, 2019, 32(9): 189-190, 192. DOI: 10.3969/j.issn.1006-1959.2019.09.065.

LIU Y Y, ZENG W J, XU X Y, et al . Postnatal thrombotic thrombocytopenic purpura: a case report and literature review[J ] . Med Inf, 2019, 32(9): 189-190, 192. DOI: 10.3969/j.issn.1006-1959.2019.09.065.

姜薇, 王慧智, 卢秋阳, 等. 产后并发血栓性血小板减少性紫癜1例[J]. 牡丹江医学院学报, 2020, 41(3): 133-134, 167. DOI: 10.13799/j.cnki.mdjyxyxb.2020.03.034.

JIANG W, WANG H Z, LU Q Y, et al . Postnatal thrombotic thrombocytopenicpurpura: a case report[J ] . J Mudanjiang Med Univ, 2020, 41(3): 133-134, 167. DOI: 10.13799/j.cnki.mdjyxyxb.2020.03.034.

TSUDA M, SHIRATSUCHI M, NAKASHIMA Y, et al . Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome[J ] . Transfus Apher Sci, 2018, 57(6): 790-792. DOI: 10.1016/j.transci.2018.10.023.

SENANAYAKE H M, PATABENDIGE M. Two potentially lethal conditions of probable immune origin occurring in a pregnant woman: a case report[J]. J Med Case Rep, 2018, 12(1): 158. DOI: 10.1186/s13256-018-1701-4.

ZHANG J, BAUGH L, GUILEYARDO J, et al . Thrombotic thrombocytopenic purpura with Graves' disease during pregnancy[J ] . Proc (Bayl Univ Med Cent), 2020, 33(2): 270-272. DOI: 10.1080/08998280.2020.1713029.

ROTTENSTREICH A, KALISH Y, TVITO A, et al . Acquired thrombotic thrombocytopenic purpura in pregnancy: The role of placental and breast-milk mediated transfer of ADAMTS13-autoantibodies[J ] . Thromb Res, 2017, 156: 80-81. DOI: 10.1016/j.thromres.2017.06.005.

STRAUBE L E, DE RIDDER G G, HUBER C A, et al . Spinal anesthetic in a patient with a platelet count of 7000×10 9 /L and undiagnosed thrombotic thrombocytopenic purpura: a case report[J ] . A A Pract, 2020, 14(6): e01184. DOI: 10.1213/XAA.0000000000001184..

ARZUAGA-MENDEZ J, MORENO M, MATEOS-MAZÓN J J, et al . Acquired thrombotic thrombocytopenic purpura diagnosed during first trimester of pregnancy with excellent outcome after plasma exchange and rituximab, a case report[J ] . Clin Case Rep, 2021, 9(3): 1304-1306. DOI: 10.1002/ccr3.3752.

AMINIMOGHADDAM S, AFROOZ N, NASIRI S, et al . A COVID-19 pregnant patient with thrombotic thrombocytopenic purpura: a case report[J ] . J Med Case Rep, 2021, 15(1): 104. DOI: 10.1186/s13256-020-02577-5.

李玲霞, 吕小慧, 刘朵朵, 等. 妊娠合并血栓性血小板减少性紫癜4例临床分析[J]. 中国妇幼健康研究, 2021, 32(4): 597-602.

LI L X, LYU X H, LIU D D, et al . Clinical analysis of 4 cases of pregnancy complicated with thrombotic thrombocytopenic purpura[J ] . Chin J Woman Child Heal Res, 2021, 32(4): 597-602.

DE SOUZA JR V R, DE OLIVEIRA A B C, VANDERLEI A M, et al . Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report[J ] . J Med Case Rep, 2018, 12(1): 15. DOI: 10.1186/s13256-017-1545-3.

杨新军, 林静, 汪晶华, 等. 剖宫产后TTP致妊娠相关急性肾损伤一例经验与教训[J]. 临床误诊误治, 2018, 31(1): 34-37. DOI: 10.3969/j.issn.1002-3429.2018.01.012.

YANG X J, LIN J, WANG J H, et al . Experiences and lessons of one postpartum thrombotic thrombocytopenic purpura with hemorrhage lead to preg-nancy-related acute kidney injury[J ] . Clin Misdiagn Misther, 2018, 31(1): 34-37. DOI: 10.3969/j.issn.1002-3429.2018.01.012.

GEORGE J N, NESTER C M, MCINTOSH J J. Syndromes of thrombotic microangiopathy associated with pregnancy[J]. Hematol Am Soc Hematol Educ Program, 2015, 2015: 644-648. DOI: 10.1182/asheducation-2015.1.644.

FAKHOURI F, SCULLY M, PROVÔT F, et al . Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group[J ] . Blood, 2020, 136(19): 2103-2117. DOI: 10.1182/blood.2020005221.

CINES D B, LEVINE L D. Thrombocytopenia in pregnancy[J]. Blood, 2017, 130(21): 2271-2277. DOI: 10.1182/blood-2017-05-781971.

中华医学会血液学分会血栓与止血学组. 血栓性血小板减少性紫癜诊断与治疗中国专家共识(2012年版)[J]. 中华血液学杂志, 2012, 21(11): 983-984. DOI: 10.3760/cma.j.issn.0253-2727.2012.11.025.

Group of Thrombosis and Hemostasis of Hematology Branch of Chinese Medical Association. Diagnosis and treatment of thrombotic thrombocytopenic purpura in China(2012)[J]. Chin J hematol, 2012, 21(11): 983-984. DOI: 10.3760/cma.j.issn.0253-2727.2012.11.025.

MATSUMOTO M, FUJIMURA Y, WADA H, et al . Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan[J ] . Int J Hematol, 2017, 106(1): 3-15. DOI: 10.1007/s12185-017-2264-7.

ZHENG X L, VESELY S K, CATALAND S R, et al . ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura[J ] . J Thromb Haemost, 2020, 18(10): 2486-24 95. DOI: 10.1111/jth.15006.

唐加明. 妊娠合并血栓性血小板减少性紫癜[J]. 中华产科急救电子杂志, 2015, 4(1): 10-14. DOI: 10.3877/cma.j.issn.2095-3259.2015.01.003.

TANG J M. Thrombotic thrombocytopenic purpura in pregnancy[J]. Chin J Obstet Emerg Electron Ed, 2015, 4(1): 10-14. DOI: 10.3877/cma.j.issn.2095-3259.2015.01.003.

ZHENG X L, VESELY S K, CATALAND S R, et al . ISTH guidelines for treatment of thrombotic thrombocytopenic purpura[J ] . J Thromb Haemost, 2020, 18(10): 2496-2502. DOI: 10.1111/jth.15010.

COPPO P, VEYRADIER A, French Reference Center for Thrombotic Microangiopathies(CNR-MAT). TTP in the setting of pregnancy: The story still has to be written[J]. J Thromb Haemost, 2020, 18(10): 2775-2777. DOI: 10.1111/jth.15030.

MARINS L R, DA ROCHA OPPERMANN M L. Thrombotic thrombocytopenic purpura and acquired immunodeficiency syndrome diagnosed in pregnancy: Case report[J]. J Obstet Gynaecol Res, 2021, 47(5): 1898-1902. DOI: 10.1111/jog.14717.

SCULLY M, STARKE R, LEE R, et al . Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpura[J ] . Blood Coagul Fibrinolys, 2006, 17(6): 459-463. DOI: 10.1097/01.mbc.0000240918.65306.20.

ROTTENSTREICH A, DOR S, KEREN-POLITANSKY A, et al . Pregnancy and non-pregnancy related immune thrombotic thrombocytopenic purpura in women of reproductive age[J ] . J Thromb Thrombolys, 2021, 51(1): 187-193. DOI: 10.1007/s11239-020-02133-4.

Views

下载量

CSCD

Alert me when the article has been cited

提交

Tools

Publicity Resources

Clinical application of plasma exchange combined with early continuous renal replacement therapy in patients with multiple injuries and high myoglobinemia

Anesthetic management in cesarean section in women with heart disease: analysis of 96 cases

Analysis of pregnancy outcomes in 267 nulliparous women after high-intensity focused ultrasound ablation of uterine fibroids

Analysis of perinatal outcomes in 232 adolescent primiparas

Application and preliminary evaluation of nutritional support in acute pancreatitis of pregnancy

Related Author

ZHANG Yuansong

ZHU Haoran

DENG Wenjun

LI Chaojun

LIU Han

REN Hongbing

Yan LIANG

Xiaohang BAO

Related Institution

Department of Emergency Medicine， First Affiliated Hospital， Army Medical University （Third Military Medical University）

Department of Anesthesia, Second Affiliated Hospital, Army Medical University (Third Military Medical University)

Key Laboratory of Ultrasound Engineering in Medicine Co-Founded by Chongqing and the Ministry of Science and Technology, Chongqing Collaborative Innovation Center for Minimally-invasive and Noninvasive Medicine, College of Biomedical Engineering, Chongqing Medical University

Ultrasound Ablation Center, the First Affiliated Hospital of Chongqing Medical University

Department of Obstetrics and Gynecology, Southwest Hospital, Third Military Medical University

⁰