Outcome of Surgical Interventions and Deliveries in Patients with Bleeding of Unknown Cause: An Observational Study

Veen, Caroline S B; Huisman, Elise J.; Romano, Lorenzo; Schipaanboord, Celesta W. A.; Cnossen, Marjon H.; Maat, Moniek P.M. de; Leebeek, Frank W.G.; Kruip, Marieke J.H.A.

doi:10.1055/s-0041-1726344

Cited by 13 publications

(8 citation statements)

References 35 publications

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“…In total three PPH (650–1500 ml) were recorded. Veen et al described a cohort of BDUC patients and pregnancy outcomes 23 . A total of 26 pregnancies were reported; 2/26 received hemostatic prophylaxis with TXA.…”

Section: Discussionmentioning

confidence: 99%

“…Veen et al described a cohort of BDUC patients and pregnancy outcomes. 23 A total of 26 pregnancies were reported; 2/26 received hemostatic prophylaxis with TXA. PPH was reported in 1/2 women who had TXA and 11/24 in those who did not have hemostatic prophylaxis and risk factors for PPH (e.g., uterine atony) were present in the majority of women, which is similar to our findings.…”

Section: Ta B L Ementioning

confidence: 99%

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Outcomes and management of pregnancy in women with bleeding disorder of unknown cause

Castle

Desborough

Kemp

et al. 2022

Journal of Thrombosis and Haemostasis

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Background: Patients with a bleeding tendency with normal laboratory tests have been described as having an unclassified bleeding disorder or bleeding disorder of unknown cause (BDUC). There are very little data available on how to manage pregnancy. Objectives:To study management and outcomes of these patients at four United Kingdom hemophilia comprehensive care centers.Methods: Retrospective case note review from 2010-2020.Results: Sixty deliveries in 36 patients were recorded. The median International Society on Thrombosis and Haemostasis bleeding assessment tool score was 9. In 54 cases for which data were available, the odds ratio for post partum hemorrhage (PPH) was 6.3 for no primary hemostatic prophylaxis versus prophylaxis (95% confidence interval 1.2-34.2, p < .05); 7/9 (78%) versus 16/45 (36%) PPH incidence for the groups, respectively. Hemostatic prophylaxis was with tranexamic acid but some patients received desmopressin or platelet infusions. Secondary PPH was seen in 5/60 (8%) of cases. No neonatal bleeding complications or maternal thromboembolic complications were noted. Avoidance of regional anesthesia and fetal delivery precautions were commonly advised, but in the small number of cases in which they occurred no complications were noted.Conclusions: Despite hemostatic prophylaxis PPH was commonly seen. Further prospective studies of BDUC patients are required to determine optimal management in pregnancy as well as determine the pathophysiological basis of bleeding. K E Y W O R D Sbleeding disorder of unknown cause, bleeding of unknown cause, post partum hemorrhage, pregnancy, tranexamic acid, unclassified bleeding disorder

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Section: Discussionmentioning

confidence: 99%

Section: Ta B L Ementioning

confidence: 99%

Outcomes and management of pregnancy in women with bleeding disorder of unknown cause

Castle

Desborough

Kemp

et al. 2022

Journal of Thrombosis and Haemostasis

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“…Furqan et al 5 described half‐dose (0.15 mcg/kg) desmopressin in combination with tranexamic acid in a small series of BUC cases. Veen et al 6 described 53 BUC patients who underwent invasive procedures. Of these patients, 4/29 covered with hemostatic prophylaxis developed major bleeding versus 6/24 who were not managed with hemostatic prophylaxis.…”

Section: Resultsmentioning

confidence: 99%

“…[1][2][3] Previously, four cases series describing management of UBD patients undergoing invasive procedures has been published with successful use of tranexamic acid and/or desmopressin; however, the quality of evidence is very low. 2,[4][5][6][7] In this study the United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO; the network of National Health Service [NHS] hospitals that manages patients with bleeding disorders) UBD working party aimed to investigate registration patterns for UBD and clinical practice via a survey of member hemophilia centers to give contemporary real-world data within this area. The working party is composed of hematology clinical scientists and hematologists from within Great Britain and Ireland.…”

Section: Introductionmentioning

confidence: 99%

Current practice and registration patterns among United Kingdom Haemophilia Centre Doctors’ Organisation centers for patients with unclassified bleeding disorders

Thomas

Downes

Evans

et al. 2021

Journal of Thrombosis and Haemostasis

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Background Bleeding of unknown cause (BUC) and unclassified bleeding disorders (UBD) are increasingly recognized. There is no guidance on diagnosis and management. Objectives To examine UK haemophilia centre registration patterns and current practice for UBD patients. Methods In a two‐step process, the UK National Haemophilia Database (NHD) was reviewed for registration patterns of UBD patients and a survey of UK haemophilia centers was conducted (January/February 2021) to capture current practice for diagnosis and management of patients with UBD. Results/discussion Overall, registrations with the NHD for UBD patients has sharply risen from 2012 to 2020 and in 2019 accounted for 2.65% of registered patients. For the survey, the response rate was 52/67 (78%). Practice was widely variable; 35/52 (67%) centers register UBD; among these 35 centers, terminology included UBD (28 centers), undiagnosed bleeding disorder (four centers), and BUC (three centers); 34/52 (65%) centers use a formal bleeding assessment tool. For management of dental extraction and high bleeding risk surgery in a fictional UBD patient we found that tranexamic acid was widely used; however, beyond this a variety of hemostatic products were advised including blood products, recombinant factor VIIa/prothrombin complex concentrate, and desmopressin. There was general consensus (≈90%) on avoiding regional anesthesia in pregnancy, but no agreement on the need for fetal precautions to avoid bleeding at delivery (50% would advise these). There was a disparity of opinion on chemical thromboprophylaxis, and management of patients without prior hemostatic challenges and offspring of these patients. Conclusion This study provides a snapshot of current practice and real‐world data in this area. Future studies need to address the gaps in evidence.

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“…[9][10][11] Patients with MBDs, particularly those with BDUC, face a risk of recurrent bleeding, as demonstrated by prospective data from the Vienna Bleeding Biobank (VIBB) and other research cohorts. [12][13][14] In fact, a majority of BDUC patients have persistent mild bleeding symptoms, including epistaxis and easy bruising, alongside an elevated risk for more severe bleeding events following surgery, tooth extraction, or during postpartum hemorrhage (PPH). While prophylactic treatments involving desmopressin (DDAVP) and/or tranexamic acid (TXA) have shown some efficacy in reducing bleeding complications, a substantial cohort of patients continues to experience bleeding episodes.…”

Section: Introductionmentioning

confidence: 99%

Bleeding Disorder of Unknown Cause: A Diagnosis of Exclusion

Mehic,

Gebhart,

Pabinger

2024

Hamostaseologie

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Patients with an unexplained mild to moderate bleeding tendency are diagnosed with bleeding disorder of unknown cause (BDUC), a classification reached after ruling out other mild to moderate bleeding disorders (MBD) including von Willebrand disease (VWD), platelet function defects (PFDs), coagulation factor deficiencies (CFDs), and non-hemostatic causes for bleeding. This review outlines our diagnostic approach to BDUC, a diagnosis of exclusion, drawing on current guidelines and insights from the Vienna Bleeding Biobank (VIBB). According to guidelines, we diagnose VWD based on VWF antigen and/or activity levels ≤50 IU/dL, with repeated VWF testing if VWF levels are <80 IU/dL. This has been introduced in our clinical routine after our findings of diagnostically relevant fluctuations of VWF levels in a high proportion of MBD patients. PFDs are identified through repeated abnormalities in light transmission aggregometry (LTA), flow cytometric mepacrine fluorescence, and glycoprotein expression analysis. Nevertheless, we experience diagnostic challenges with regard to reproducibility and unspecific alterations of LTA. For factor (F) VIII and FIX deficiency, a cutoff of 50% is utilized to ensure detection of mild hemophilia A or B. We apply established cutoffs for other rare CFD being aware that these do not clearly reflect the causal role of the bleeding tendency. Investigations into very rare bleeding disorders due to hyperfibrinolysis or increase in natural anticoagulants are limited to cases with a notable family history or distinct bleeding phenotypes considering cost-effectiveness. While the pathogenesis of BDUC remains unknown, further explorations of this intriguing area may reveal new mechanisms and therapeutic targets.

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Outcome of Surgical Interventions and Deliveries in Patients with Bleeding of Unknown Cause: An Observational Study

Cited by 13 publications

References 35 publications

Outcomes and management of pregnancy in women with bleeding disorder of unknown cause

Outcomes and management of pregnancy in women with bleeding disorder of unknown cause

Current practice and registration patterns among United Kingdom Haemophilia Centre Doctors’ Organisation centers for patients with unclassified bleeding disorders

Bleeding Disorder of Unknown Cause: A Diagnosis of Exclusion

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