Clinical characteristics of women captured by extending the definition of severe postpartum haemorrhage with 'refractoriness to treatment': a cohort study

Background: The absence of a uniform and clinically relevant definition of severe postpartum haemorrhage hampers comparative studies and optimization of clinical management. The concept of persistent postpartum haemorrhage, based on refractoriness to initial first-line treatment, was proposed as an alternative to common definitions that are either based on estimations of blood loss or transfused units of packed red blood cells (RBC). We compared characteristics and outcomes of women with severe postpartum haemorrhage captured by these three types of definitions. Methods: In this large retrospective cohort study in 61 hospitals in the Netherlands we included 1391 consecutive women with postpartum haemorrhage who received either ≥4 units of RBC or a multicomponent transfusion. Clinical characteristics and outcomes of women with severe postpartum haemorrhage defined as persistent postpartum haemorrhage were compared to definitions based on estimated blood loss or transfused units of RBC within 24 h following birth. Adverse maternal outcome was a composite of maternal mortality, hysterectomy, arterial embolisation and intensive care unit admission. Results: One thousand two hundred sixty out of 1391 women (90.6%) with postpartum haemorrhage fulfilled the definition of persistent postpartum haemorrhage. The majority, 820/1260 (65.1%), fulfilled this definition within 1 h following birth, compared to 819/1391 (58.7%) applying the definition of ≥1 L blood loss and 37/845 (4.4%) applying the definition of ≥4 units of RBC. The definition persistent postpartum haemorrhage captured 430/471 adverse maternal outcomes (91.3%), compared to 471/471 (100%) for ≥1 L blood loss and 383/471 (81.3%) for ≥4 units of RBC. Persistent postpartum haemorrhage did not capture all adverse outcomes because of missing data on timing of initial, first-line treatment. Conclusion: The definition persistent postpartum haemo

Identification of Self-Limiting Pediatric Immune Thrombocytopenia at Diagnosis

Stemcel biology/Regenerative medicine (incl. bloodtransfusion

THE OCCURRENCE AND IMPACT OF JOINT BLEEDS IN VON WILLEBRAND DISEASE

Clinical epidemiolog

The occurrence and impact of joint bleeds in Von Willebrand disease

Background: Von Willebrand disease (VWD) is a heterogeneous inherited bleeding disorder that affects up to 1% of the population. Joint bleeds are not predominant, but have been reported to occur in 8-45% of patients with VWD, especially in those with a more severe phenotype. Joint bleeds can lead to structural joint damage. The most severe type 3 VWD patients develop similar rates of joint range of motion limitation over time as moderate hemophilia A patients. However, the severity, onset and impact of joint bleeds and its complications in VWD patients are largely unknown. Aims: The aim of this study is to assess the incidence, onset and treatment of joint bleeds and its impact on quality of life and joint integrity in moderate and severe VWD. Methods: In the Willebrand in the Netherlands (WIN) study 804 moderate and severe VWD patients (VWF activity ≤30U/dL) completed a comprehensive questionnaire after giving informed consent. We quantified joint bleed occurrence as reported in the questionnaires and examined the medical files for documentation on joint bleeds and joint problems from all patients who had reported treatment for joint bleeds with desmopressin or clotting factor concentrate (CFC) and from as many patients without joint bleeds for comparison, matched for gender, age, FVIII and VWF activity. Results: Twenty three percent of the patients (184/804) self-reported joint bleeds in the questionnaire, mostly in the knee, followed by the ankle and elbow. These patients had more severe VWD (type 3 VWD 12% vs. 4%, p50% in 14/16 VWD patients with joint bleeds who started CFC prophylaxis. We found documented X-ray joint damage in 44% of the patients with joint bleeds compared to 11% of the controls (

Time between inhibitor detection and start of immune tolerance induction: Association with outcome in the BrazIT study

Background Immune tolerance induction (ITI) is the treatment of choice for eradication of anti-factor VIII (FVIII) neutralizing alloantibodies (inhibitors) in people with inherited hemophilia A and high-responding inhibitor (PwHA-HRi). The association between ITI outcome and time elapsed between inhibitor detection and start of ITI ( increment t(inhi-ITI)) is debatable. Objective The aim of this study was to evaluate this association among a large cohort of severe PwHA-HRi. Methods Severe (factor VIII activity level 0.6-1.7 year), third (>1.7-9.2 years), and fourth quartile (>9.2-24.5 years). The overall success rate was 65.5% (93/142), with no difference among first, second, third, and fourth quartiles (62.9%, 69.4%, 58.3%, and 71.4%, respectively) even after adjusting the analyses for potential confounders. Conclusion In conclusion, delayed ITI start is not associated with failure of ITI in PwHA-HRi. Therefore, ITI should be offered for these patients, regardless of the time elapsed between the detection of inhibitor and the ITI start.Clinical epidemiolog