天疱疮诱导获得性血友病 A 患者发生危及生命的出血，并用利妥昔单抗和 rFVIIa 成功治疗：一例报告。
Life-threatening bleeding in a patient with pemphigoid-induced acquired hemophilia A and successfully treated with rituximab and rFVIIa: A case report.
机构信息
Department of Hematology, West China Hospital, Sichuan University, Chengdu 610041, China.
出版信息
Medicine (Baltimore). 2021 Jan 22;100(3):e24025. doi: 10.1097/MD.0000000000024025.
RATIONALE
Acquired hemophilia A (AHA) is a rare bleeding disorder with prolonged activated partial thromboplastin time (aPTT). Severe hemorrhage may occur, especially in refractory AHA.
PATIENT CONCERNS
We reported a 63-year-old man who suffered from life-threatening bleeding after the onset in lower limbs.
DIAGNOSES
The patient was diagnosed as AHA which was related to pemphigoid.
INTERVENTIONS
The patient had no response to the first-line treatment with corticosteroid and cyclophosphamide. Meanwhile, fatal hemorrhage occurred successively in thoracic cavity and right frontal lobe. rFVIIa and rituximab were administered.
OUTCOMES
The patient survived from the life-threatening hemorrhage with a normal aPTT. His aPTT and FVIII:C level was normal during the follow-up of 6 months.
LESSONS
Rituximab and rFVIIa can play a critical role in rescuing AHA that is refractory to the first-line treatment.
背景
获得性血友病 A（AHA）是一种罕见的出血性疾病，伴有延长的活化部分凝血活酶时间（aPTT）。可能会发生严重出血，尤其是在难治性 AHA 中。
病例报告
我们报告了一例 63 岁男性患者，下肢发病后出现危及生命的出血。
诊断
患者被诊断为与天疱疮相关的 AHA。
干预措施
该患者对一线治疗（皮质类固醇和环磷酰胺）无反应。同时，胸腔和右额叶相继发生致命性出血。给予 rFVIIa 和利妥昔单抗。
结果
患者从危及生命的出血中存活下来，aPTT 正常。在 6 个月的随访期间，他的 aPTT 和 FVIII：C 水平正常。
结论
利妥昔单抗和 rFVIIa 可在挽救一线治疗无效的 AHA 中发挥关键作用。
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