
诊断学理论与实践››2024,Vol. 23››Issue (05): 524-530.doi:10.16150/j.1671-2870.2024.05.009
收稿日期:2024-04-08接受日期:2024-10-08出版日期:2024-10-25发布日期:2025-02-25通讯作者:孙梅 E-mail:smzgwj@126.comQIAN Lingling, PEI Xiaoping, SUN Aihong, HEI Bin, SUN Mei(
)
Received:2024-04-08Accepted:2024-10-08Published:2024-10-25Online:2025-02-25摘要:
目的:探讨获得性血友病A(Acquired hemophilia A,AHA)的病因、临床表现、诊治及预后,提高临床对AHA的认识及诊治水平。方法:对我院2013年1月至2023年12月诊断的7例AHA患者的进行回顾性分析,并分别以“Acquired hemophilia A”及“获得性血友病A”在PubMed及中国知网、万方数据库中检索相关文献,从一般资料、病因、诊断时间、临床表现、治疗及预后方面进行对比分析。结果:7例AHA患者均为女性,中位年龄为44.1岁(26~68岁),1例继发于乙型病毒性肝炎感染,1例继发于妊娠,1例为消化道手术后发病,1例继发于干燥综合征。皮肤瘀斑是AHA患者最主要临床表现(7/7),且均有活化部分凝血活酶时间(activated partial thromboplastin time, APTT)升高、凝血因子Ⅷ(factor Ⅷ, FⅧ)活性(FⅧ:C)降低、FⅧ抗体(FⅧ:Ab)升高。有6例患者接受止血治疗,所有患者接受清除抗体治疗。本组中位随访391 d(28~777 d),除1例患者失访,4例患者达到完全缓解(complete response, CR),达到CR的中位时间为40 d(27~48 d),1例患者达到部分缓解(partial response, PR),均未出现复发。1例患者治疗无效(no response, NR),在随访期间无新发临床出血表现。文献检索到1252例,AHA男女均可发病,男女比例为1.18:1,男女中位年龄分别为65.6岁、55.8岁。56.8% AHA患者为特发型,11.4%与肿瘤、11.1%与自身免疫性疾病、6.7%与妊娠等相关。55%患者接受旁路途径药物是止血治疗,清除抗体治疗多采用以糖皮质激素、环磷酰胺及利妥昔单抗为基础的方案。结论:AHA可继发于肿瘤、自身免疫性疾病、感染、妊娠等,患者多有皮肤淤斑;旁路途径止血、免疫抑制治疗清除抗体,多数患者预后良好。
中图分类号:
钱玲玲, 裴孝平, 孙爱红, 何斌, 孙梅. 单中心7例获得性血友病A的回顾性临床研究[J]. 诊断学理论与实践, 2024, 23(05): 524-530.
QIAN Lingling, PEI Xiaoping, SUN Aihong, HEI Bin, SUN Mei. A single-center retrospective clinical study of 7 cases of acquired hemophilia A[J]. Journal of Diagnostics Concepts & Practice, 2024, 23(05): 524-530.
表1
7例获得性血友病A患者的一般资料
| Case | 1 | 2 | 3 | 4 | 5 | 6 | 7 |
|---|---|---|---|---|---|---|---|
| Sex | Female | Female | Female | Female | Female | Female | Female |
| Age(y) | 26 | 29 | 32 | 50 | 68 | 62 | 42 |
| Time from onset to diagnosis(d) |
7 | 18 | 25 | 24 | 9 | 10 | 14 |
| Pathogen | HBV | Pregnancy | - | - | - | Surgery | SS |
| Hemorrhagic manifestations | Ecchymosis | Ecchymosis | Ecchymosis | Ecchymosis | Ecchymosis | Ecchymosis | Ecchymosis |
| Joint bleeding | Soft tissue hemorrhage | Muscle hematoma | Muscle hematoma | Gastrointestinal bleeding | Gastrointestinal bleeding | ||
| Urinary tract bleeding | Joint bleeding | Soft tissue hemorrhage | |||||
| Gums bleeding | |||||||
| Surgical area bleeding |
表2
7例获得性血友病A患者的初诊时检查结果、治疗及转归
| Indice | Case | ||||||
|---|---|---|---|---|---|---|---|
| 1 | 2 | 3 | 4 | 5 | 6 | 7 | |
| APTT(s) | 141 | 124 | 83.7 | 92.8 | 101.9 | 128 | 124 |
| FⅧ:C(%) | 1 | 0.30 | 0.67 | 1 | 1 | 0.17 | 0.15 |
| FⅧ:Ab(BU) | 20 | 13.28 | 6.6 | 0.92 | 0.62 | 50 | 87.5 |
| Hemostasis | - | PCC | PCC | PC | Plasma | TXA | Plasma |
| rFⅦa | FⅧ | FⅧ | TXA | cryoprecipitation | cryoprecipitation | ||
| PCC | |||||||
| FⅧ | |||||||
| rFⅦa | |||||||
| IST | IGIV | IGIV | Pred | Pred | Pred | Pred | Pred |
| Pred | CTX | CTX | RTX | CTX | |||
| RTX | |||||||
| Outcome | Lost to follow-up | CR | CR | CR | CR | NR | PR |
| Onset time(d) | - | 48 | 40 | 27 | 45 | - | 42 |
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