scholarly journals Outcomes in Patients With Hemophilia and von Willebrand Disease Undergoing Invasive or Surgical Procedures

2016 ◽  
Vol 23 (2) ◽  
pp. 148-154 ◽  
Author(s):  
John Chapin ◽  
Jaqueline Bamme ◽  
Fraustina Hsu ◽  
Paul Christos ◽  
Maria DeSancho
Keyword(s):  
Retrospective Chart Review ◽  
Adverse Outcomes ◽  
Von Willebrand Disease ◽  
Interdisciplinary Management ◽  
Invasive Procedures ◽  
Acute Bleeding ◽  
Management Guidelines ◽  
Delayed Bleeding ◽  
Dental Procedures ◽  
Von Willebrand

Adults with hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD) frequently require surgery and invasive procedures. However, there is variability in perioperative management guidelines. We describe our periprocedural outcomes in this setting. A retrospective chart review from January 2006 to December 2012 of patients with HA, HB, and VWD undergoing surgery or invasive procedures was conducted. Type of procedures, management including the use of continuous factor infusion, and administration of antifibrinolytics were reviewed. Adverse outcomes were defined as acute bleeding (<48 hours), delayed bleeding (≥48 hours), transfusion, inhibitor development, and thrombosis. We identified 59 patients with HA and HB. In all, 24 patients had severe hemophilia and 12 had mild/moderate hemophilia. Twelve patients had inhibitors. There were also 5 female carriers of HA and 6 patients with VWD. There were 34 major surgeries (26 orthopedic, 8 nonorthopedic) and 129 minor surgeries. Continuous infusion was used in 55.9% of major surgeries versus 8.5% of minor surgeries. Antifibrinolytics were administered in 14.7% of major surgeries versus 23.2% of minor surgeries. In all, 4 patients developed acute bleeding and 10 patients developed delayed bleeding. Delayed bleeding occurred in 28.6% of genitourinary procedures and in 16.1% of dental procedures. Five patients acquired an inhibitor and 2 had thrombosis. In conclusion, patients with HA, HB, or VWD had similar rates of adverse outcomes when undergoing minor surgeries or major surgeries. This finding underscores the importance of an interdisciplinary management and procedure-specific guidelines for patients with hemophilia and VWD prior to even minor invasive procedures.

Outcomes In Adult Hemophilia and Von Willebrand Disease Patients Undergoing Surgical and Invasive Procedures

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1120-1120
Author(s):  
John C. Chapin ◽  
Jacqueline Bamme ◽  
Fraustina Hsu ◽  
Paul Christos ◽  
Maria Teresa De Sancho
Keyword(s):  
Tertiary Care ◽  
Von Willebrand Disease ◽  
Major Surgery ◽  
Care Hospital ◽  
Invasive Procedures ◽  
Acute Bleeding ◽  
Inhibitor Development ◽  
Delayed Bleeding ◽  
Dental Procedures ◽  
Von Willebrand

Abstract Introduction Adults with hemophilia and von Willebrand disease (VWD) frequently require surgical and invasive procedures. There is variability in current practice of peri-operative management in major surgery and in the management of invasive procedures. The purpose of this study was to evaluate outcomes and management strategies in patients with hemophilia and VWD undergoing surgical and invasive procedures in a comprehensive hemophilia center at a tertiary care hospital. Methods A retrospective review of electronic medical records was carried out from patients with hemophilia and VWD seen at the Weill Cornell Hemophilia Treatment Center undergoing surgery or an invasive procedure from January 2006 to December 2012. Information on demographics, diagnosis, severity, and presence of inhibitors was also collected. Procedures and treatment strategies were reviewed including the type of procedure, use of factor bolus/DDAVP, continuous infusion(CI), and antifibrinolytics. Outcomes from these cases were reviewed for acute bleeding (<48 hours after procedure), delayed bleeding (>48 hours after procedure), transfusion of blood products, inhibitor development, and post-procedure thrombosis. Data were compared using Fischer’s exact test. Results Our study population consisted of 59 patients (mean age=58, range 21-77). Our hemophilia group included 41 with hemophilia A and 7 with hemophilia B. Our hemophilia patients were severe (n=24), mild-mod (n=12), and 12 had inhibitors. We also identified 5 female carriers of hemophilia A.We identified 6 VWD patients including Type 1 (n=3), 2A (n=1), 3 (n=2). We reviewed 34 major surgeries (26 orthopedic, 8 non-orthopedic) and 91 invasive procedures. We covered all patients an initial dose of either factor VIII/IX or DDAVP. In addition, 55.9% of major procedures were covered by continuous factor infusion (CI), whereas only 9.9% of minor procedures were covered by CI. Conversely, antifibrinolytics were used in 14.7% of major surgeries and 34.1% of minor procedures. Antifibrinolytics were used in all types of minor procedures except those involving the genitourinary tract. Hematologic outcomes are shown in Table 1. We identified 4 cases of acute bleeding and 10 cases of delayed bleeding. We also identified 5 cases of inhibitor development. We identified one thrombotic episode in an orthopedic surgery case. We further examined several classes of procedures including dental, GI endoscopies, GU procedures, biopsies, and GYN procedures in carriers. One transfusion was needed in the case of a liver biopsy; acute bleeding was noted in 2 biopsies and 1 endoscopy. Delayed bleeding accompanied 28.6% of GU procedures and 16.1% of dental procedures. Inhibitor development was noted after 1 GU procedure and 2 dental procedures. Conclusions Our adult hemophilia and VWD population undergoing minor procedures showed similar rates of adverse hematologic outcomes compared to major surgery. This finding highlights the significant risk of even minor procedures in adults with bleeding disorders. Appropriate treatment standards should be designed to take into account patient responses, procedure type, and anticipated outcomes. Disclosures: No relevant conflicts of interest to declare.

Patterns of Bleeding in Males with Von Willebrand Disease

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1129-1129
Author(s):  
Manpreet K. Sandhu ◽  
Shailja Shah ◽  
Sari H Jacoby ◽  
Alice J. Cohen
Keyword(s):  
Family History ◽  
Positive Family History ◽  
Von Willebrand Disease ◽  
Bleeding Complications ◽  
Invasive Procedures ◽  
Dental Procedures ◽  
The Mean ◽  
Von Willebrand ◽  
Male Subjects

Abstract Abstract 1129 Background: Von Willebrand disease (vWD) is the most common inherited bleeding disorder in the United States, affecting about 1–2 % of the population. It is generally inherited as an autosomal dominant trait and is equally prevalent in males and females. Though this disorder usually manifests as mucosal bleeding, it is well known that there is a wide variation in clinical severity. The symptoms of vWD are usually more obvious in women because of menstruation and labor/delivery; and may be significantly under recognized in men. The pattern of bleeding and presentation at diagnosis in males has previously only been described in a small population of type 1 vWD patients (pts). Patients and Methods: We performed a retrospective chart review of active male pts with vWD receiving care at the Comprehensive Hemophilia Treatment Center. Clinical information such as age at diagnosis, initial symptom prompting the diagnosis and clinical bleeding history was obtained. Invasive procedures were reviewed for bleeding complications and relevant hematological intervention. Relevant laboratory data like PT, PTT, von Willebrand antigen (vWF:Ag), von Willebrand ristocetin cofactor (vWF:RCoF) and factor VIII activity (FVIII:C) was noted. Results: We identified a total of 140 male subjects with the diagnosis of vWD. Of the 140, 85 were evaluable with complete data. The mean age of the pts at the time of the study is 19 years (yrs) (1–79 yrs). 76/85 (89%) are type 1, 6/85 (7%) type 2 and 2/85 (4%) type 3 vWD. The mean age at diagnosis was 9.7 yrs (0.5–60 yrs) with 62/85 (73%) being diagnosed at age ≤ 10 yrs. The most common initial presentations leading to the diagnosis of vWD were epistaxis (25/85; 29%), a positive family history (24/85; 28%), prolonged preoperative (preop) PTT (14/85; 16%), easy bruising (10/85; 12%) and postoperative (postop) bleeding (5/85; 6%) pts. The mean lab values of vWF:Ag 48 IU/dl, vWF:RCoF 45 IU/dl and FVIII:C 62 IU/dl. 25/85 (29%) pts have one of the above parameters measuring < 30 IU/dl. Among those who ever experienced bleeding, the most common manifestations were epistaxis (30/85; 35%), easy bruising (10/85; 12%), postop bleeding (10/85; 8%), hemarthrosis (4/85; 5%), hematuria and oral bleeds (3/85; 4% each). A total of 48 surgical procedures occurred, consisting of 18 dental procedures, 13 tonsillectomy/adenoidectomy (T/A), 7 circumcisions, 5 joint surgeries, 2 port placements and 1 each of cystoscopy, orchiopexy and heart surgery. Bleeding complications were common 10/48 (21%): 5 with dental procedures, 2 each with T/A and circumcision and 1 with cystoscopy; all occurring in the absence of prophylactic treatment and 2/10 (20%) complications requiring transfusion of blood products. Conclusions: In our study which included types 1, 2 and 3 vWD pts, the majority of male subjects were symptomatic at the time of diagnosis, whereas about 40% were brought to attention due to a positive family history or an abnormal preop screening test. As opposed to previous reports, postop bleeding was a rare initial presentation in our larger cohort, possibly due to improved preop screening and earlier diagnosis. The most common pattern of bleeding amongst the symptomatic pts was mucosal, in the form of epistaxis and easy bruising. Bleeding complications can occur with invasive procedures, so aggressive use of DDAVP or clotting factor concentrates is warranted in a pt with known diagnosis of vWD. Disclosures: No relevant conflicts of interest to declare.

Practical aspects of DDAVP use in patients with von Willebrand Disease undergoing invasive procedures: a European survey

Haemophilia ◽  
2015 ◽  
Vol 22 (1) ◽  
pp. 110-120 ◽  
Author(s):  
J. Windyga ◽  
G. Dolan ◽  
C. Altisent ◽  
O. Katsarou ◽  
M.-F. López Fernández ◽  
...  
Keyword(s):  
Von Willebrand Disease ◽  
Invasive Procedures ◽  
European Survey ◽  
Von Willebrand

Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study

Blood ◽  
2002 ◽  
Vol 99 (2) ◽  
pp. 450-456 ◽  
Author(s):  
Pier M. Mannucci ◽  
Juan Chediak ◽  
Wahid Hanna ◽  
John Byrnes ◽  
Marlies Ledford ◽  
...  
Keyword(s):  
Factor Viii ◽  
Von Willebrand Factor ◽  
High Purity ◽  
Bleeding Time ◽  
Von Willebrand Disease ◽  
Invasive Procedures ◽  
Bleeding Episodes ◽  
Type 3 ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract Among patients with von Willebrand disease (VWD) who are unresponsive to desmopressin therapy, replacement with plasma-derived concentrates is the treatment of choice. Because prospective studies are lacking, such treatment has been largely empirical. A multicenter, prospective study has been conducted in 81 patients with VWD (15 patients with type 1, 34 with type 2, and 32 with type 3 disease) to investigate the efficacy of a high-purity factor VIII/von Willebrand factor (FVIII/VWF) concentrate for treatment of bleeding and surgical prophylaxis. Two preparations of the concentrate—one virally inactivated with solvent detergent, the other with an additional heat-treatment step—were evaluated. Pharmacokinetic parameters were similar for both preparations. Using pre-established dosages based on the results of pharmacokinetic studies, 53 patients were administered either preparation for the treatment of 87 bleeding episodes, and 39 patients were treated prophylactically for 71 surgical or invasive procedures. Sixty-five (74.7%) and 10 (11.5%) of the bleeding episodes were controlled with 1 or 2 infusions, respectively. Patients with severe type 3 VWD typically required more infusions and higher doses, at shorter time intervals, than did patients with generally milder types 1 and 2. Among patients undergoing surgical procedures, blood loss was lower than that predicted prospectively, and losses exceeding the predicted value did not correlate with the postinfusion skin bleeding time. In conclusion, the concentrate effectively stopped active bleeding and provided adequate hemostasis for surgical or invasive procedures, even in the absence of bleeding time correction.

Practical aspects of factor concentrate use in patients with von Willebrand disease undergoing invasive procedures: a European survey

Haemophilia ◽  
2016 ◽  
Vol 22 (5) ◽  
pp. 739-751 ◽  
Author(s):  
J. Windyga ◽  
G. Dolan ◽  
C. Altisent ◽  
O. Katsarou ◽  
M.-F. López Fernández ◽  
...  
Keyword(s):  
Von Willebrand Disease ◽  
Invasive Procedures ◽  
European Survey ◽  
Factor Concentrate ◽  
Von Willebrand

scholarly journals Von Willebrand disease in pregnancy: a case report

2021 ◽  
Vol 10 (12) ◽  
pp. 4602
Author(s):  
Rashmi A. G. ◽  
Riya Kumar ◽  
Dayamayi A. S. ◽  
Spandana Nallapilli
Keyword(s):  
Blood Loss ◽  
Post Partum ◽  
Adverse Outcomes ◽  
Von Willebrand Disease ◽  
Clotting Factor ◽  
Medical College ◽  
Life Threatening ◽  
Von Willebrand ◽  
Willebrand Factor

Von Willebrand disease (VWD) is a hereditary bleeding disorder that can be severe and potentially life-threatening, particularly in pregnant women during labor and subsequently during early puerperium. There is no optimal treatment or management for this disorder. Hence, all efforts aim at early diagnosis and the focus is mainly on minimising and controlling blood loss. We described the case of a woman in the post-partum period with severe VWD, admitted in the obstetrics and gynaecology ward at Rajarajeswari Medical College and Hospital, Bangalore. Prompt diagnosis, initiation of pre-partum and intra-partum Von Willebrand factor (VWF)/clotting factor replacement therapy, vigilant post-partum monitoring of blood loss and systematic follow up will help expedite recovery and prevent adverse outcomes.

Efficacy of Alphanate® (Human Factor VIII/Von Willebrand Factor Concentrate) in Preventing Excessive Bleeding during Surgery or Invasive Procedures in Patients with Congenital Von Willebrand Disease. A Multicenter Retrospective Study.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3951-3951
Author(s):  
M. Elaine Eyster ◽  
Charles L. Sexauer ◽  
Simon Brown ◽  
Richard A. Lipton ◽  
Craig M. Kessler ◽  
...  
Keyword(s):  
Replacement Therapy ◽  
Rating Scale ◽  
Invasive Procedure ◽  
Von Willebrand Disease ◽  
Major Surgery ◽  
Invasive Procedures ◽  
Total N ◽  
Human Factor Viii ◽  
Minor Surgery ◽  
Von Willebrand

Abstract Objective: To assess the efficacy of Alphanate® as replacement therapy in subjects with congenital VWD undergoing surgical or invasive procedures. Methodology: Retrospective data from 5 hospitals and accounting for 8 years of chart review has been collected. The study protocol and the subject’s informed consent were approved by the local Institutional Review Boards. To date, 35 treated events (27 subjects) have been evaluated. Events were classified by local investigators and an independent referee committee as major or minor surgery, and invasive procedures. Treatment efficacy was rated using a 4-point verbal rating scale. Replacement therapy was considered effective if the treatment outcome was rated as excellent or good, and non-effective when the outcome was rated as poor or none. Results: Efficacy results (number and percentage) obtained by local investigators and the referee committee are presented in Tables 1 and 2. Conclusions: Since there may be bias in the interpretation of responses made by the participating physicians, it was felt that true responses might better be assessed retrospectively by an adjudication committee. Therefore, both Tables were presented here. Much of the discrepancies between these 2 tables was generated by oral surgery. There was a high level of efficacy agreement between investigators and the referree committee. Therefore, it is concluded that Alphanate® is effective in preventing excessive bleedings during surgeries and invasive procedures in subjects with congenital VWD. Table 1. Efficacy Results Obtained by Investigators Major surgery (n=9) Minor surgery (n=17) Invasive procedure (n=9) Total (n=35) Excellent 6 (66.7%) 14 (82.4%) 8 (88.9%) 28 (80.0%) Good 3 (33.3%) 1 (5.9%) 1 (11.1%) 5 (14.3%) Poor 0 (0.0%) 1 (5.9%) 0 (0.0%) 1 (2.9%) None 0 (0.0%) 1 (5.9%) 0 (0.0%) 1 (2.9%) Table 2. Efficacy Results Obtained by Referee Committee Major Surgery (n=12) Minor Surgery (n=11) Invasive Procedure (n=12) Total (n=35) Excellent 8 (66.7%) 11 (100%) 11 (91.7%) 30 (85.7%) Good 1 (8.3%) 0 (0.0%) 0 (0.0%) 1 (2.9%) Poor 3 (25.0%) 0 (0.0%) 1 (8.3%) 4 (11.4%) None 0 (0.0%) 0 (0.0%) 0 (0.0%) 0 (0.0%)

A Perspective on the Use of FVIII Concentrates and Cryoprecipitate Prophylactically in Surgery or Therapeutically in Severe Bleeds in Patients with von Willebrand Disease Unresponsive to DDAVP: Results of an International Survey

1995 ◽  
Vol 74 (05) ◽  
pp. 1370-1378 ◽  
Author(s):  
Paul A Foster
Keyword(s):  
Bleeding Time ◽  
Von Willebrand Disease ◽  
Clinical Settings ◽  
Primary Therapy ◽  
Bleeding Events ◽  
Dental Procedures ◽  
Post Infusion ◽  
A Prospective Study ◽  
Surgical Setting ◽  
Von Willebrand

SummaryAn international registry was established by the Subcommittee on von Willebrand Factor of the SSC/ISTH on the treatment of patients with types of von Willebrand disease (vWd) unresponsive to DDAVP infusion. Data was collected on 76 surgical events in 64 patients from 19 treatment centers. Thirty-three non-mucosal, 12 mucosal, 10 orthopedic and 21 dental procedures were reported. In the 76 surgical events, 14 cases prophylactically received cryoprecipitate while 62 received factor VIII (FVIII) concentrate. Surgical hemostasis was reported as satisfactory, good, or excellent in 75 of the 76 cases. Post-infusion bleeding times were measured in only three of 14 surgical events treated with cryoprecipitate. All three cases had a reduction but not correction of the bleeding time. The post-infusion bleeding time was measured in 27 of 62 cases in which FVIII concentrates were used. The bleeding time was normalized in 15, reduced but not normalized in eight, and not changed from baseline in four. Data was also collected from 16 treatment centers on 50 serious bleeding events in 35 patients. These included 19 gastrointestinal, 15 other mucosal, four central nervous system, seven orthopedic, and five other bleeds. Eleven cases received cryoprecipitate and 39 received FVIII concentrate as primary therapy. The efficacy of treatment was considered good or excellent in 49 of 50 cases. Post-infusion bleeding times were measured in only 15 of the 50 reported bleeding events. The post-infusion bleeding time was normalized in six, decreased but not normalized in eight, and not changed from baseline in one. In this retrospective survey, FVIII concentrates were subjectively as efficacious as cryoprecipitate in both the surgical setting and for the treatment of severe bleeds in patients with types of vWd unresponsive to DDAVP. Since the bleeding time was monitored during therapy in only a minority of these cases, a definitive relationship between the efficacy of therapy and normalization or reduction of the bleeding time in these two clinical settings cannot be established from this study. A prospective study on the use of FVIII and/or vWf concentrates in these clinical settings is necessary to address this issue.

Von Willebrand Disease in Taiwan: A Clinical and Laboratory Studies of 52 Patients.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3974-3974
Author(s):  
Yeu-Chin Chen ◽  
Tsu-Yi Chao ◽  
Shin-Nan Cheng
Keyword(s):  
Laboratory Tests ◽  
Invasive Procedure ◽  
Mean Value ◽  
Von Willebrand Disease ◽  
Type I ◽  
Excessive Bleeding ◽  
Disease Subtype ◽  
Dental Procedures ◽  
Number Of Patients ◽  
Von Willebrand

Abstract Von Willebrand disease (VWD) has been found to be the most common inherited bleeding disorder in Caucasian with a prevalence of around 1%, yet it has not been well recognized in Taiwan and Asian countries. Only a small number of patients have been identified and reported. The aim of this retrospective study was to identify patients with VWD by clinical manifestation and laboratory tests in Taiwan. VWD was detected by a panel of laboratory tests, including bleeding time, aPTT, factor VIII activity assay, Von Willebrand antigen (VWF: Ag) and ristocetin cofactor activity (VWF: RCo), and platelet function analyzer (PFA) test using both Collagen/ADP and Collagen/Epinephrine (CEPI). VWF multimer analysis was performed by western blot to confirm the disease subtype. From October, 2003 to April, 2007, 52 of 494 (9.5%) patients, from 44 families, were identified to have VWD, including 16 men and 36 women. Their median age was 29 with a range of 4∼69 years of age. The most frequently encountered reasons for VWD detection was menorrhagia accounting for 19.2%. The others included inherited detection because of affected family (11.5%), prolonged aPTT (11.5%), excessive bleeding after dental procedures (9.6%) and excessive bleeding after surgery or invasive procedure (9.6%). By laboratory tests, the mean value of VWF: Ag and VWF: RCo was 46.9 ± 18.4% and 32.3 ± 17.4%, respectively. The most sensitive test was VWF: RCo with 90.3%, followed by CEPI with 83.8%. Of 37 patients with VWF multimer analysis, 34(92%) were revealed to have type I VWD, 1 had type IIA, and 2 from the same family had type III VWD. In conclusion, our study demonstrated that VWD was not an uncommon inherited bleeding disease in Taiwan. Menorrhagia was the most common reason for VWD determination, and VWF: RCo and PFA test CEPI were the more sensitive tests for the VWD identification.

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