Acquired hemophilia as an obstetric and interdisciplinary challenge

Abstract

Summary. Introduction. Unexplained postpartum bleeding refractory to standard hemostatic measures should raise a high clinical suspicion for an acquired bleeding disorder, for instance postpartum acquired hemophilia (РАН). Aim: present the clinical and laboratory characteristics and treatment outcomes in patients with PAH. Materials and Methods. The single-center retrospective non-randomized cohort study included 20 women with an established diagnosis of PAH, who were followed up at the National Medical Research Center for Hematology from 2008 to 2024. An analysis of their clinical and laboratory data considered medical history features, management strategy, and specific therapy outcomes. Results. The time to onset of clinical manifestations in most patients (n = 18; 90%) was from 0 to 4 months (median — 1.5 months) after delivery. The debut of hemorrhagic syndrome in 13 (65%) women was observed after the first delivery, in 5 (25%) — after the second delivery, and in 2 (10%) — after the third delivery. At the time of diagnosis, the clinical picture was characterized by severe bleeding in 13 (65%) of 20 women and mild bleeding in 7 (35%), respectively. The disease phenotype was characterized by hematomas of various sites in 15 (75%) and uterine bleeding in 10 (50%) patients; nasal, gingival bleeding, hematuria, and hemarthrosis were less common. A simultaneous combination of several hemorrhagic manifestations was reported in 12 (60%) women. A higher median titer of the blood coagulation factor VIII (FVIII) inhibitors was recorded among patients with severe hemorrhagic syndrome (51 BU/ml vs. 4.9 BU/ml; p = 0.034). In the compa- rison groups the medians of activated partial thromboplastin time and activity of the blood coagulation factor FVIII did not differ significantly by the severity of bleeding (p > 0.05). Disease remission was achieved in 12 (66.7%) of 18 dynamically examined patients: 5 (27.8%) patients achieved spontaneous remission and 7 (38.9%) — therapeutic one. Relapse of acquired hemophilia was recorded in 1 case during a subsequent pregnancy. Conclusion. The demonstrated clinical and laboratory characteristics and treatment outcomes in patients with PAH in the Russian population, may be useful to physicians who encounter acquired coagulopathies for the first time in their clinical practice.

For citation: Surenkov А.А., Yakovleva Е.V., Orel Е.В., Dvirnyk V.N., Zozulya N.I. Acquired hemophilia as an obstetric and interdisciplinary challenge. Tromboz, gemostaz i reologiya. 2026;(1):45–55. (In Russ.).

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