Introduction: Postpartum haemorrhage (PPH) is the major cause of maternal death worldwide. Haemostatic abnormalities are independently associated with a significantly increased risk for severe PPH. In this study, the value of haemostatic evaluation in women with severe PPH was explored. Aim: To investigate the occurrence of previously unknown inherited bleeding disorders in women with severe PPH. Methods: Women with severe PPH (blood loss of ≥2000 mL) between 2011 and 2017, referred to the haematology outpatient clinic for haemostatic evaluation, were retrospectively included. A bleeding disorder was diagnosed based on (inter)national guidelines, or when having a clear bleeding phenotype, not fulfilling any diagnostic criteria or laboratory abnormalities, this being classified as Bleeding of Unknown Cause (BUC). Logistic regression was used to model the association between diagnosis and obstetrical causes and risk factors for PPH. Results: In total, 85 women with PPH were included. In 23% (n = 16), a mild bleeding disorder was diagnosed, including low von Willebrand factor (Low VWF 8/16), platelet function disorders (PFD 5/16), BUC (2/16) and von Willebrand disease type 1 (1/16). No significant associations were found between obstetrical causes or risk factors for PPH and the presence of a bleeding disorder. Conclusion: In 23% of women with severe PPH, a mild bleeding disorder was diagnosed, independent of obstetrical causes or risk factors for PPH. This implies that severe PPH can be the first clinical symptom of an inherited bleeding disorder. Therefore, to optimize clinical management, haemostatic evaluation after severe PPH is recommended.

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Keywords haemostasis, inherited blood coagulation disorders, postpartum haemorrhage, von Willebrand disease, women's health
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Series VSNU Open Access deal
Journal Haemophilia
Veen, C.S.B. (Caroline S. B.), van der Reijken, I.S. (Irene S.), Jansen, A.J.G, Schipaanboord, C.W.A. (Celesta W. A.), Visser, W. (Willy), de Maat, M.P.M, … Kruip, M.J.H.A. (2019). Severe postpartum haemorrhage as first presenting symptom of an inherited bleeding disorder. Haemophilia. doi:10.1111/hae.13846