Service and Central Laboratory of Haematology, Department of Oncology and Department of Laboratories and Pathology, Lausanne University Hospital (CHUV), University of Lausanne (UNIL), Lausanne, Switzerland.
Department of Haematology and Central Haematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Haemophilia. 2021 Mar;27(2):199-210. doi: 10.1111/hae.14233. Epub 2021 Feb 6.
About 1%-5% of acquired haemophilia A cases affect mothers in the postpartum setting.
This study delineates the characteristics of this disease, specific to the postpartum setting, notably relapse in subsequent pregnancies.
Report of two cases and literature study (1946-2019), yielding 73 articles describing 174 cases (total 176 cases).
Patients were aged 29.9 years (17-41) and 69% primigravidae. Diagnosis was made at a median of 60 days after delivery (range 0-308). Bleeding types were obstetrical (43.4%), cutaneous (41.3%), and muscular (36.7%). In >90% of the cases, FVIII at diagnosis was <1% (range 0%-8%). FVIII inhibitor was documented in 75.4% cases (median titre of 20 BU/ml, range 1-621). Haemostatic treatment was necessary in 57.1% using fresh frozen plasma (16%), factor concentrate (27.6%) and/or bypassing agents (37.4%). Immunosuppressive treatment was administered in 90.8%, mostly steroids (85.3%), alone or combined with immunosuppressants (27%). Rituximab was used mostly as a second line treatment. Only 24 patients (13.6%) had documented subsequent pregnancies and 6 (22.2%) suffered haemophilia recurrence during pregnancy.
This study allows better definition of: (1) clinical and laboratory characteristics of postpartum acquired haemophilia, (2) response to therapy, and (3) the risk of relapse for subsequent pregnancies.
约 1%-5%的获得性血友病 A 病例发生于产后的母亲。
本研究阐述了产后发病的获得性血友病 A 的特征，尤其是随后妊娠时的复发。
报告两例病例并进行文献研究（1946-2019 年），共纳入 73 篇文章中描述的 174 例病例（共计 176 例）。
患者年龄为 29.9 岁（17-41 岁），69%为初产妇。中位诊断时间为产后 60 天（范围 0-308 天）。出血类型为产科（43.4%）、皮肤（41.3%）和肌肉（36.7%）。90%以上的病例 FVIII 在诊断时<1%（范围 0%-8%）。75.4%的病例有 FVIII 抑制剂（中位数效价 20BU/ml，范围 1-621）。57.1%的患者需要进行止血治疗，采用新鲜冰冻血浆（16%）、因子浓缩物（27.6%）和/或旁路制剂（37.4%）。90.8%的患者接受了免疫抑制治疗，主要为类固醇（85.3%），单独或联合免疫抑制剂（27%）。利妥昔单抗主要作为二线治疗药物。仅有 24 例患者（13.6%）有后续妊娠记录，6 例（22.2%）在妊娠期间出现血友病复发。
本研究更好地定义了：（1）产后获得性血友病的临床和实验室特征，（2）治疗反应，以及（3）随后妊娠时的复发风险。
