9例自身免疫性疾病合并获得性血友病A的回顾性临床分析

高飞; 田淼; 李光; 杜明珠; 周智辉; 宋艳萍; 董昌虎

doi:10.16766/j.cnki.issn.1674-4152.002677

9例自身免疫性疾病合并获得性血友病A的回顾性临床分析

doi: 10.16766/j.cnki.issn.1674-4152.002677

高飞^{1, 2},
田淼¹,
李光²,
杜明珠²,
周智辉¹,
宋艳萍²,
董昌虎^1, ,

1.
陕西中医药大学第二临床医学院，陕西咸阳 712046
2.
西安交通大学附属西安市中心医院西安市血液病研究所，陕西西安 710003

基金项目:

陕西省自然科学基础研究计划项目 2019JM-607

陕西省教育厅专项科研计划项目 20JK0604

详细信息

通讯作者:
董昌虎, E-mail: DCH819@sina.com

中图分类号: R593.2R544.1
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- 文章访问数: 133
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- PDF下载量: 7
- 被引次数: 0
出版历程
- 收稿日期: 2022-02-10
- 网络出版日期: 2022-11-30

A retrospective clinical study of 9 cases with autoimmune diseases combined acquired hemophilia A

1.
The Second Clinical Medical College, Shaanxi University of Chinese Medicine, Xianyang, Shaanxi 712046, China

摘要

摘要: 目的分析并总结自身免疫性疾病合并获得性血友病A患者的临床特征、实验室检查结果、治疗方案及疗效，提高对疾病的认识。方法选择西安市中心医院2012年1月—2020年12月收治的9例自身免疫性疾病合并获得性血友病A的患者，回顾性分析其临床特征、实验室检查结果、治疗方案及疗效。结果 9例患者中，男性4例，女性5例，中位年龄为60.0(42.0~72.5)岁。出血症状存在异质性，以皮肤出血为主，也可出现消化道出血及黄体出血等重要脏器出血。关于清除抑制物的治疗，糖皮质激素单药应用4例(44.5%)，环磷酰胺联合泼尼松(CP)方案1例(11.1%)，利妥昔单抗联合CP方案或环磷酰胺、长春新碱与泼尼松(COP)方案3例(33.3%)，环磷酰胺单药应用1例(11.1%)。其中完全缓解3例(33.3%)，部分缓解4例(44.5%)，未缓解2例(22.2%)，总有效率为77.8%。结论自身免疫性疾病合并获得性血友病A，起病急，出血较重且存在异质性。采用免疫抑制疗法有效率较高，部分病例在免疫抑制治疗的基础上联合应用利妥昔单抗也可取得较好的疗效。经免疫抑制及补充凝血因子治疗可控制病情，疗效较好，但44.5%的患者经过1个疗程治疗后仅达到部分缓解，提示要彻底清除抑制物，需要针对原发病进行系统治疗，同时需要多疗程或较长时间的免疫抑制治疗。
- 获得性血友病A /
- 回顾性分析 /
- 免疫抑制剂 /
- 糖皮质激素 /
- 利妥昔单抗
Abstract: Objective To analyze and summarize the clinical features, laboratory examination results, treatment plan and curative effects of patients with autoimmune diseases combined with acquired hemophilia A, so as to improve the understanding of the disease. Methods Nine patients with autoimmune diseases combined acquired hemophilia A who were admitted by Xi ' an Central Hospital from January 2012 to December 2020 were selected, and their clinical features, laboratory examination results, treatment plans and curative effects were retrospectively analyzed. Results Among the 9 patients, 4 were males and 5 were females. The median age was 60.0 (42.0~72.5) years. Bleeding symptoms were heterogeneous, mainly skin bleeding, but also bleeding from important organs such as gastrointestinal bleeding and corpus luteum hemorrhage. In the treatment of elimination of inhibitors, glucocorticoid monotherapy was used in 4 cases (44.5%), cyclophosphamide and prednisone (CP) regimen in 1 case (11.1%), rituximab combined with CP regimen or cyclophosphamide, vincristine and prednisone (COP) regimen in 3 cases (33.3%), and cyclophosphamide monotherapy in 1 case (11.1%). Among them, 3 cases were complete remission (33.3%), 4 cases were partial remission (44.5%), and 2 cases (22.2%) were not remission, and the total effective rate was 77.8%. Conclusion Autoimmune diseases combined acquired hemophilia A have a rapid onset, heavy bleeding and heterogeneity. The use of immunosuppressive therapy is more effective. In some cases, the combination of rituximab on the basis of immunosuppressive therapy can also achieve better results. After immunosuppression and coagulation factor supplementation, the disease can be quickly controlled and the effect is good, but 44.5% of patients only achieved partial remission after one course of treatment, suggesting that to completely remove the inhibitors, systemic treatment is required for the primary disease, and multiple courses of treatment or longer-term immunosuppressive treatment are required.
- Acquired hemophilia A /
- Retrospective analysis /
- Immunosuppressant /
- Glucocorticoid /
- Rituximab

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表 1 9例AHA患者的临床资料

Table 1. Clinical data of 9 patients with AHA

项目	例数(%)
性别
男性	4(44.4)
女性	5(55.6)
合并疾病
类风湿性关节炎	1(11.1)
干燥综合征	1(11.1)
未分化结缔组织病	3(33.3)
桥本甲状腺炎	1(11.1)
类天疱疮	2(22.2)
系统性红斑狼疮	1(11.1)

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表 2 9例AHA患者的实验室检查结果、治疗方案与疗效

Table 2. Laboratory test results, treatment plans and curative effects of 9 patients with AHA

序号	FⅧ：C(%)	抑制物(BU/mL)	治疗前APTT(s)	清除抑制物	止血治疗	疗效
1	3.7	1.8	62.60	R-COP	PCC/FⅧ	CR
2	2.3	7.6	77.70	R-CP	PCC/FFP	CR
3	31.0	阳性	60.70	Pred	FFP	NR
4	1.0	阳性	120.20	CTX	PCC/FⅧ	NR
5	2.1	阳性	54.00	DXM	FFP	CR
6	1.4	7.2	105.40	CP	PCC/FFP	PR
7	9.2	4.6	53.10	MP	FFP/Cryo	PR
8	1.9	阳性	57.00	Pred	PCC/FFP	PR
9	2.3	阳性	80.70	R-CP	PCC/FFP	PR

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