Postpartum Hemorrhage In Women With Von Willebrand Disease and Other Bleeding Disorders

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2357-2357
Author(s):  
Lynn M. Malec ◽  
Margaret V. Ragni ◽  
Charity G. Moore ◽  
Jonathan Yabes ◽  
Jie Li
Keyword(s):  
At Risk ◽  
Caesarean Section ◽  
Pregnancy Complications ◽  
Postpartum Hemorrhage ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Medical Comorbidities ◽  
Live Births ◽  
Caesarean Section Delivery ◽  
Von Willebrand

Abstract Background Postpartum hemorrhage (PPH) is a cause of potentially serious morbidity and mortality in women of reproductive age. Prior data suggests that women with von Willebrand Disease (VWD) and other inherited bleeding disorders (OBD) experience PPH at a rate greater than the general population. Little published data exist regarding the incidence or predictors of PPH in women with VWD or OBD to guide obstetricians and hematologists in the management of these at-risk women. Methods In order to evaluate the incidence of PPH, clinical characteristics and obstetric outcomes of women with VWD and other inherited bleeding disorders we analyzed data from the Pennsylvania Health Care Cost Containment Council (PHC4) database between January 1, 2007 and December 31, 2011. The council collects data from over 4.5 million inpatient hospital discharges and ambulatory/outpatient procedure records each year from hospitals and freestanding ambulatory surgery centers in Pennsylvania. Data from all women with VWD and OBD (including factor XI deficiency, hemophilia A carriers, hemophilia B carriers) as well as all women who experienced PPH was extracted from the database. Data elements, in addition to demographic factors, were identified by ICD-9 codes, including medical comorbidities, hospital length of stay, obstetric complications, and birth outcomes. We compared data from women with VWD and OBD, specifically in those with and without PPH. Differences between groups were analyzed using chi-square or Fisher’s exact tests for categorical variables and two-sample t-tests for continuous variables. Results Between 2007 and 2011, there were a total of 1,234 women with bleeding disorders, including 506 with VWD and 728 with OBD, who underwent delivery. Of these, 54 (4.4%) experienced PPH, including 28 (5.5%) with VWD and 26 (3.6%) with OBD. Among women with VWD, those with PPH were younger, 25.4 vs. 27.8 years (p=0.034), and nearly 2-fold more likely to have anemia, 27.6% vs. 14.2% (p=0.059), but had no greater frequency of caesarean section delivery (p=0.234) or pregnancy complications (p=0.678), including antepartum bleeding, preeclampsia, placental abruption, gestational diabetes, fetal growth restriction, preterm labor, or intrauterine fetal death. There were no differences in live births or mortality (all p≥0.999), nor in medical comorbidities (p=0.300), including diabetes, hypertension, obesity, and hyperlipidemia. Among women with OBD, those with PPH were over 5-fold more likely to have anemia, 48.1% vs. 8.6% (p<0.001), but had no higher rates of caesarean section delivery (p=0.234) or pregnancy complications, mortality or lower rates of live births (all p≥0.999). Discussion The incidence of postpartum hemorrhage (PPH) in women with VWD is 5.5% and 3.6% in those with OBD. Importantly, anemia appears to be a marker of PPH in women with bleeding disorders as it is present in over one-fourth (27.6%) of women with VWD and PPH and in nearly one-half (48.1%) of those with OBD. These data suggest that screening hemoglobin, along with standard screening of factor levels in late pregnancy, may help identify those with VWD or OBD at risk for PPH. Future prospective studies will be needed to confirm this hypothesis. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Resolving Differential Diagnostic Problems in von Willebrand Disease, in Fibrinogen Disorders, in Prekallikrein Deficiency and in Hereditary Hemorrhagic Telangiectasia by Next-Generation Sequencing

Life ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 202
Author(s):  
Réka Gindele ◽  
Adrienne Kerényi ◽  
Judit Kállai ◽  
György Pfliegler ◽  
Ágota Schlammadinger ◽  
...  
Keyword(s):  
Next Generation Sequencing ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Von Willebrand Disease ◽  
Differential Diagnostics ◽  
Bleeding Disorders ◽  
Next Generation ◽  
Illumina Platform ◽  
Clinical Patient ◽  
Von Willebrand ◽  
Generation Sequencing

Diagnosis of rare bleeding disorders is challenging and there are several differential diagnostics issues. Next-generation sequencing (NGS) is a useful tool to overcome these problems. The aim of this study was to demonstrate the usefulness of molecular genetic investigations by summarizing the diagnostic work on cases with certain bleeding disorders. Here we report only those, in whom NGS was indicated due to uncertainty of diagnosis or if genetic confirmation of initial diagnosis was required. Based on clinical and/or laboratory suspicion of von Willebrand disease (vWD, n = 63), hypo-or dysfibrinogenemia (n = 27), hereditary hemorrhagic telangiectasia (HHT, n = 10) and unexplained activated partial thromboplastin time (APTT) prolongation (n = 1), NGS using Illumina platform was performed. Gene panel covered 14 genes (ACVRL1, ENG, MADH4, GDF2, RASA1, F5, F8, FGA, FGB, FGG, KLKB1, ADAMTS13, GP1BA and VWF) selected on the basis of laboratory results. We identified forty-seven mutations, n = 29 (6 novel) in vWD, n = 4 mutations leading to hemophilia A, n = 10 (2 novel) in fibrinogen disorders, n = 2 novel mutations in HHT phenotype and two mutations (1 novel) leading to prekallikrein deficiency. By reporting well-characterized cases using standardized, advanced laboratory methods we add new pieces of data to the continuously developing “bleeding disorders databases”, which are excellent supports for clinical patient management.

Coagulopathies

2020 ◽  
Author(s):  
Michael Levine
Keyword(s):  
Key Words ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Coagulation Cascade ◽  
Bleeding Disorders ◽  
Platelet Disorders ◽  
Von Willebrand

Coagulopathy can be caused by numerous hereditary or acquired etiologies. Although some of these conditions are known and the patient is aware of the bleeding disorder, other bleeding disorders are diagnosed only after the onset of excessive hemorrhage. This review discusses both hereditary and acquired disorders of coagulopathy. Platelet disorders are discussed elsewhere. This review contains 2 figures, 7 tables, and 72 references. Key words: Coagulopathies; Coagulopathy; Bleeding disorder; Hereditary bleeding disorder; Acquired bleeding disorder; von Willebrand disease; Hemophilia; Coagulation cascade; Hemorrhage; Anticoagulant-associated hemorrhage

scholarly journals Desmopressin: a nontransfusional form of treatment for congenital and acquired bleeding disorders [see comments]

Blood ◽  
1988 ◽  
Vol 72 (5) ◽  
pp. 1449-1455 ◽  
Author(s):  
PM Mannucci
Keyword(s):  
Arginine Vasopressin ◽  
Von Willebrand Disease ◽  
Synthetic Analogue ◽  
Bleeding Disorders ◽  
Spinal Fusion Surgery ◽  
Prolonged Bleeding Time ◽  
Normal Individuals ◽  
Transfusion Requirements ◽  
Coagulant Activity ◽  
Von Willebrand

Abstract Desmopressin (1-deamino-8-D-arginine vasopressin, abbreviated DDAVP) is a synthetic analogue of the antidiuretic hormone L-arginine vasopressin. Because it can raise circulating levels of factor VIII coagulant activity (FVIII) and von Willebrand factor and shorten the prolonged bleeding time, DDAVP is established as a nontransfusional form of treatment for mild and moderate hemophilia and von Willebrand disease. Recently, DDAVP has also been purported to be useful for shortening the prolonged skin bleeding times that occur with uremia, cirrhosis, and platelet dysfunctions of various etiologies. Finally, there is evidence from controlled clinical trials that DDAVP can reduce blood loss and transfusion requirements for hemostatically normal individuals undergoing spinal fusion surgery and for patients undergoing cardiopulmonary bypass surgery. The purpose of this report is to review the therapeutic applications of DDAVP in congenital and acquired bleeding disorders and to discuss areas in which further basic and clinical research is needed.

Management Strategies of Stroke and Acute Coronary Syndrome In Von Willebrand Disease and Hemophilia: Experience From a Case Series and a Swiss Survey of Frequencies.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1406-1406 ◽  
Author(s):  
Sara C. Meyer ◽  
Elina Armstrong ◽  
Christine Perdrizat ◽  
Hans-Rudolf Schmid ◽  
Riitta Lassila ◽  
...  
Keyword(s):  
Acute Coronary Syndrome ◽  
Ct Scan ◽  
Treatment Strategies ◽  
Case Series ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Coronary Syndrome ◽  
Federal Statistical ◽  
Swiss Population ◽  
Von Willebrand

Abstract Abstract 1406 Introduction: Thrombolysis and combined antiplatelet therapy is considered contraindicated in patients (pts) with bleeding disorders such as von Willebrand disease (VWD) and hemophilia (HP) presenting with stroke or acute coronary syndrome (ACS). Conservative treatment e.g. of stroke is often unsatisfactory in pts within the time window for thrombolysis. As stroke and ACS are common disorders, they may coincide with prevalent bleeding disorders such as VWD and HP and increase in frequency due to growing life expectancy in VWD and HP. An emergency protocol for safe management is desirable. Methods: We report on the management of 5 Caucasians with known VWD or HP suffering from stroke or ACS. In addition we screened the Swiss population of VWD and HP patients over 10y for the occurrence of stroke or ACS based on the data of the Swiss Federal Statistical Office. Case Series. Pt 1 is a 54-y-old Swiss female suffering from mild VWD 1 with a relevant bleeding history and characteristic VWF multimers (Tab 1). She presented with acute stroke with right hemiparesis (NIHSS 9) 2h after onset. CT scan excluded intracranial bleeding. Substitution of VWF/FVIII concentrate aiming at 80% of VWF activity resulted in 55% VWF ristocetin cofactor activity (RCo) and 76% VWF antigen (Ag). Concomitant thrombolysis with full-dose rtPA was performed. After 24h NIHSS improved to 6, RCo was 45%, Ag 63% and no signs of bleeding occurred. Secondary prophylaxis with aspirin was started. She fully recovered after 3 months. Pt 2, a 68-y-old Finnish female with VWD 2A presented with a minor stroke with mild left hemiparesis and a fall (NIHSS 2) 2h after onset. CT scan was normal and she was treated with dipyridamole. Symptoms regressed to NIHSS 0 after 6h. Pt 3 to 5 had ACS. Pt 3, a 73-y-old Swiss male with hemophilia B was successfully treated for unstable angina by percutaneous coronary intervention (PCI) with stenting at a subtotal stenosis of the RCA after substitution of FIX to 65%. Postinterventionally he was on clopidogrel for 2y and then switched to aspirin. Concomitant weekly substitution of FIX led to FIX peaks of ≂f55% and nadir of ≂f15%, was then reduced in frequency and stopped after 15 months. No bleeding complications occurred. Pt 4, a 67-y-old Finnish female with symptomatic VWD 3 presented with a NSTEMI and was successfully treated without PCI or platelet inhibition. Pt 5, a 70-y-old Finnish male with symptomatic VWD 2A suffered from ACS and had angiography with substitution of VWF/FVIII concentrate to 40–134% RCo and 134–252% Ag. Aortocoronary bypass surgery was planned due to 3-vessel disease. No antiplatelet therapy was started. Survey. The analysis of the Swiss Federal Statistical Office revealed 39 cases of VWD or HP with stroke or ACS over 10y (1998-2008) in the Swiss population of 7.70 millions (2008). A lower mean age at death in pts with VWD and HP (63 y) as compared to the normal population (77y), underreporting in the early years and a potential protective effect may contribute to reduced numbers. 19 pts with VWD showed 13 events of stroke and 6 of ACS while 20 hemophiliacs had 8 events of stroke and 12 of ACS. Conclusion: Our survey confirms the occurrence of stroke and ACS in pts with VWD and HP. Observed treatment strategies are very heterogeneous. Intravascular mechanical intervention such as PCI appears advantageous in pts with VWD and HP. If intravascular therapy is not timely available or not possible, we suggest substitution to ≂f80% VWF immediately followed by full-dose thrombolysis as described in pt 1. Repetitive substitution of VWF on a 12–24h basis or infusion over 48h may be adequate in severe deficiencies (Blood 2009;114:5256). A FIX level of 25% may suffice for safe PCI in hemophilia B given the clinical experience with oral anticoagulation. Generally, treatment strategies for stroke and ACS in pts with VWD and HP should be individually tailored and balanced between thrombotic and bleeding risk. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Thalidomide for the treatment of gastrointestinal bleeding due to angiodysplasia in a patient with Glanzmann’s thrombasthenia

2017 ◽  
Vol 9 (2) ◽  
Author(s):  
Bruno K.L. Duarte ◽  
Silvia M. De Souza ◽  
Carolina Costa-Lima ◽  
Samuel S. Medina ◽  
Margareth C. Ozelo
Keyword(s):  
Side Effects ◽  
Low Dose ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Gi Bleeding ◽  
Glanzmann’S Thrombasthenia ◽  
Glanzmann's Thrombasthenia ◽  
Temporary Control ◽  
Von Willebrand ◽  
Common Manifestation

Angiodysplasia is a frequent cause of persistent gastrointestinal (GI) hemorrhage in elderly patients. Although GI bleeding isn’t the most common manifestation in patients with bleeding disorders, when present, it represents a challenging complication. We describe a 62-year-old patient with Glanzmann’s thrombasthenia, who used thalidomide for severe and recurrent GI bleeding. For 6 months, the patient experienced temporary control of GI bleeding with thalidomide in a daily oral dose of 100 mg. The anti-angiogenic effects of thalidomide have recently been explored by several groups, particularly in the management of bleeding from angiodysplasia, including cases with von Willebrand disease. Here, we review the relevant descriptions of the use of thalidomide in this situation, and also discuss potential reasons why we observed only a temporary control of the GI bleeding in our patient, such as the use of low-dose regimen due to limitations posed by thalidomide side effects.

scholarly journals Clinicopathological Profile of the von Willebrand Disease in a Tertiary Care Centre in Varanasi

2021 ◽  
Author(s):  
Pawan K. Pandey ◽  
Vijai Tilak ◽  
Mahima Yadav ◽  
Neelu Kashyap
Keyword(s):  
Diagnostic Tests ◽  
Tertiary Care ◽  
Von Willebrand Disease ◽  
North India ◽  
Bleeding Disorders ◽  
Tertiary Care Centre ◽  
Laboratory Features ◽  
Clinicopathological Profile ◽  
Relative Prevalence ◽  
Von Willebrand

Abstract Objective The von Willebrand disease (vWD) is one of the most common inherited bleeding disorders in India; however, the diagnostic tests and its interpretation require specialized laboratory and personnel which are not readily available in the eastern part of North India. The purpose of this study is to estimate the relative prevalence of vWD and study the clinical and laboratory features including advanced diagnostic tests. Methods All patients referred to the pathology department for evaluation of bleeding were evaluated for vWD during a period of 4 years. Clinical and laboratory features were analyzed and reported. Results A total of 1,126 cases of bleeding manifestations were evaluated, and 237 cases of inherited bleeding disorders were diagnosed; vWD was diagnosed in 38 (16%) of these 237 cases. Advanced diagnostic tests were done in all of these cases. Conclusion The vWD is among the most common inherited bleeding disorders in the country, second only to hemophilia A. Type-1 vWD was the most frequent with 25 cases (65.7%), followed by type-2N with 7 cases (18.4%).

scholarly journals Desmopressin: a nontransfusional form of treatment for congenital and acquired bleeding disorders [see comments]

Blood ◽  
1988 ◽  
Vol 72 (5) ◽  
pp. 1449-1455
Author(s):  
PM Mannucci
Keyword(s):  
Arginine Vasopressin ◽  
Von Willebrand Disease ◽  
Synthetic Analogue ◽  
Bleeding Disorders ◽  
Spinal Fusion Surgery ◽  
Prolonged Bleeding Time ◽  
Normal Individuals ◽  
Transfusion Requirements ◽  
Coagulant Activity ◽  
Von Willebrand

Desmopressin (1-deamino-8-D-arginine vasopressin, abbreviated DDAVP) is a synthetic analogue of the antidiuretic hormone L-arginine vasopressin. Because it can raise circulating levels of factor VIII coagulant activity (FVIII) and von Willebrand factor and shorten the prolonged bleeding time, DDAVP is established as a nontransfusional form of treatment for mild and moderate hemophilia and von Willebrand disease. Recently, DDAVP has also been purported to be useful for shortening the prolonged skin bleeding times that occur with uremia, cirrhosis, and platelet dysfunctions of various etiologies. Finally, there is evidence from controlled clinical trials that DDAVP can reduce blood loss and transfusion requirements for hemostatically normal individuals undergoing spinal fusion surgery and for patients undergoing cardiopulmonary bypass surgery. The purpose of this report is to review the therapeutic applications of DDAVP in congenital and acquired bleeding disorders and to discuss areas in which further basic and clinical research is needed.

scholarly journals Genetic and Bioinformatic Strategies to Improve Diagnosis in Three Inherited Bleeding Disorders in Bogotá, Colombia

Genes ◽  
2021 ◽  
Vol 12 (11) ◽  
pp. 1807
Author(s):  
Juliana Lago ◽  
Helena Groot ◽  
Diego Navas ◽  
Paula Lago ◽  
María Gamboa ◽  
...  
Keyword(s):  
Data Analysis ◽  
Clinical Laboratory ◽  
Low Cost ◽  
Molecular Genetic ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Congenital Diseases ◽  
Software Analysis ◽  
Molecular Genetic Basis ◽  
Von Willebrand

Inherited bleeding disorders (IBDs) are the most frequent congenital diseases in the Colombian population; three of them are hemophilia A (HA), hemophilia B (HB), and von Willebrand Disease (VWD). Currently, diagnosis relies on multiple clinical laboratory assays to assign a phenotype. Due to the lack of accessibility to these tests, patients can receive an incomplete diagnosis. In these cases, genetic studies reinforce the clinical diagnosis. The present study characterized the molecular genetic basis of 11 HA, three HB, and five VWD patients by sequencing the F8, F9, or the VWF gene. Twelve variations were found in HA patients, four in HB patients, and 19 in WVD patients. From these variations a total of 25 novel variations were found. Disease-causing variations were used as positive controls for validation of the high-resolution melting (HRM) variant-scanning technique. This approach is a low-cost genetic diagnostic method proposed to be incorporated in developing countries. For the data analysis, we developed an accessible open-source code in Python that improves HRM data analysis with better sensitivity of 95% and without bias when using different HRM equipment and software. Analysis of amplicons with a length greater than 300 bp can be performed by implementing an analysis by denaturation domains.

Managing Preoperative Hemostasis in Patients with Inherited and Acquired Bleeding Disorders

2019 ◽  
Vol 46 (01) ◽  
pp. 017-025
Author(s):  
Patrick Kyei Mensah ◽  
Sue Pavord
Keyword(s):  
Preoperative Planning ◽  
Antiplatelet Agents ◽  
Von Willebrand Disease ◽  
Bleeding Disorders ◽  
Close Collaboration ◽  
Hemostatic Agents ◽  
Risk Of Bleeding ◽  
Patients At Risk ◽  
Von Willebrand ◽  
Attention To Detail

AbstractInherited and acquired bleeding disorders pose significant hemostatic challenges for surgery. Patients at particular risk of bleeding include those with inherited bleeding disorders such as hemophilia, von Willebrand disease, and platelet function defects; those on antiplatelet agents or anticoagulants; and those with acquired conditions such as immune thrombocytopenic purpura, liver disease, or renal impairment. Each has its own specific challenges and close collaboration between the anesthetic, surgical, and hematology teams is crucial. Optimizing surgical hemostasis for patients at risk involves attention to detail, with careful preoperative planning, meticulous surgical technique, prompt identification of complications and judicious use of hemostatic agents and blood components. This article gives an overview of the bleeding risks involved and therapeutic options to overcome them.

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