妊娠期血栓性血小板减少性紫癜2例报告及文献复习

陈晓霞; 王楠楠; 严小丽; 王丹; 常青

doi:10.16016/j.2097-0927.202109074

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妊娠期血栓性血小板减少性紫癜2例报告及文献复习

临床医学 | 更新时间：2026-01-09

- 妊娠期血栓性血小板减少性紫癜2例报告及文献复习
- Thrombotic thrombocytopenicpurpura during pregnancy: report of 2 cases and literature review
- 陆军军医大学学报 2022年44卷第4期页码：390-封3
- 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：
  
  [严小丽，E-mail: yanxl1982@qq.com YAN Xiaoli, E-mail: yanxl1982@qq.com
- 基金信息：
- DOI：10.16016/j.2097-0927.202109074
  中图分类号： R554.6;R714.254
- 收稿：2021-09-08，
  
  修回：2021-11-16，
  
  纸质出版：2022-02-28
- 稿件说明：
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陈晓霞, 王楠楠, 严小丽, 等. 妊娠期血栓性血小板减少性紫癜2例报告及文献复习[J]. 陆军军医大学学报, 2022,44(4):390-封3.

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.
陈晓霞, 王楠楠, 严小丽, 等. 妊娠期血栓性血小板减少性紫癜2例报告及文献复习[J]. 陆军军医大学学报, 2022,44(4):390-封3. DOI： 10.16016/j.2097-0927.202109074.

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

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