获得性血栓性血小板减少性紫癜2例及文献复习
| 作者: |
1吴娜,
2齐林,
2成志勇,
1赵亚玲,
3潘玉夏,
4王素云
1 定州市人民医院血液内科，河北 定州 073000 2 保定市第一医院血液内科，河北 保定 071000 3 河北医科大学第二医院血液内科，石家庄 050000 4 深圳市龙华区中心医院血液内科，广东 深圳 518000 |
| 通讯: |
王素云
Email: suyunwcn@163.com |
| DOI: | 10.3978/j.issn.2095-6959.2021.10.039 |
| 基金: | 河北省医学科学研究计划(20170100)。 |
摘要
获得性血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura，TTP)发病凶险，病死率极高，早期容易误诊。本文回顾性分析2例TTP患者的临床资料及诊治经过并复习相关文献。患者均为中年女性，均存在血小板减少、微血管病性溶血性贫血、神经系统症状，血管性血友病因子裂解蛋白酶(vWF-cp/ADAMTS13)活性极度降低(<10%)，抑制物阳性。在给予血浆置换、糖皮质激素和/或利妥昔单抗治疗后，患者病情恢复。获得性TTP诊断依赖临床特征及ADAMTS13活性重度减低、抑制物阳性而确诊，治疗以血浆置换联合糖皮质激素和/或利妥昔单抗等，积极控制原发病是获得性TTP抢救成功并减少复发的关键。
关键词：
紫癜；血栓性血小板减少性；血管性血友病因子裂解蛋白酶；血浆置换
Acquired thrombotic thrombocytopenic purpura: Two case reports and literature review
CorrespondingAuthor: WANG Suyun Email: suyunwcn@163.com
DOI: 10.3978/j.issn.2095-6959.2021.10.039
Foundation: This work was supported by the Medical Science Research Program of Hebei Province, China (20170100).
Abstract
Acquired thrombotic thrombocytopenic purpura (TTP) is dangerous with a high mortality rate and it is easy to be misdiagnosed early. This paper retrospectively analyzed the clinical data, diagnosis and treatment of 2 patients with TTP, and reviewed the relevant literature. They were middle-aged women with thrombocytopenia, microangiopathic hemolytic anemia, nervous system symptoms, and extremely low activity of vWF-CP /ADAMTS13 (<10%) with positive inhibitory substance. After treatment with plasmapheresis, glucocorticoids, and/or rituximab, the patient recovered. The diagnosis of acquired TTP depends on the clinical characteristics, severe reduction of ADAMTS13 activity and positive inhibitory substance, and the treatment of plasma exchange combined with glucocorticoid and/or rituximab. Active control of primary disease is the key to successful rescue of acquired TTP and reduce recurrence.
Keywords：
purpura; thrombotic thrombocytopenic; ADAMTS13; plasmapheresis