scholarly journals An Unexpected Cause of Catastrophic Bleeding: A Case Report

2020 ◽  
Vol 26 (1) ◽  
pp. 44-46
Author(s):  
Majid H. Alabbood
Keyword(s):  
Preoperative Assessment ◽  
Thyroid Function Test ◽  
Massive Bleeding ◽  
Thyroid Stimulating Hormone ◽  
Bleeding Tendency ◽  
History Taking ◽  
Laboratory Findings ◽  
Acquired Von Willebrand Syndrome ◽  
Moderate Disease ◽  
Von Willebrand

Profound hypothyroidism has been linked to mild bleeding diathesis such as easy bruising and menorrhagia. Bleeding tendency depends on the severity of hypothyroidism. Those with moderate disease are prone to thrombotic events, while those with profound hypothyroidism are prone to bleeding. This paper reports a case of severe intraoperative bleeding during elective rhinoplasty in a patient with missed profound hypothyroidism. The clinical and laboratory findings are presented with a review of the literature. A 43-year-old female was admitted for an elective rhinoplasty. In the theater, the patient developed massive bleeding at the beginning of surgery. On the next day, the patient was discharged home for further assessment with a massive bruise all over her face. Upon history taking, the patient denied any drug abuse or previous bleeding episode or family history of bleeding disorder. Surprisingly, a transverse scar was noted at the lower part of the neck. The patient admitted that she had thyroidectomy done 2 years ago, and she was kept on thyroxine replacement and stopped it by herself 18 months ago. Thyroid-stimulating hormone (TSH) was 70 mU/L, and von Willebrand factor antigen/ristocetin cofactor was normal. A diagnosis of acquired von Willebrand syndrome type 1 was made. The patient was kept on thyroxine 150 µg/day. Six weeks later, TSH was 0.8 mU/L, and all bleeding parameters were corrected. A careful history taking and general examination looking for hypothyroidism is crucial in the preoperative assessment. It might be prudent to include thyroid function test in the routine preoperative investigation of all patients.

Von-Willebrand-Syndrom Typ 1 und Schwangerschaft

2011 ◽  
Vol 31 (S 01) ◽  
pp. S11-S13 ◽  
Author(s):  
J. Oppermann ◽  
A. Siegemund ◽  
R. Schobess ◽  
U. Scholz
Keyword(s):  
Clinical Presentation ◽  
Bleeding Disorder ◽  
Bleeding Tendency ◽  
Laboratory Findings ◽  
Mucous Membranes ◽  
Von Willebrand

SummaryThe von Willebrand-Jürgens syndrome (VWJS) type 1 is a common hereditary bleeding disorder with a bleeding tendency located especially in the mucous membranes. Women suffering from VWJS type 1 show menorrhagia and prolonged postoperative bleedings. During pregnancy the clinical presentation varies by the increase of the von Willebrand factors.In this article the laboratory findings and the clinical presentation of patients with VWJS during pregnancy was examined. The necessity of interventions during pregnancy and at the time of delivery was under consideration.

scholarly journals Clinical Significance of Inhibitors in Acquired von Willebrand Syndrome

Blood ◽  
1998 ◽  
Vol 91 (10) ◽  
pp. 3623-3629 ◽  
Author(s):  
Hiroshi Mohri ◽  
Shigeki Motomura ◽  
Heiwa Kanamori ◽  
Michio Matsuzaki ◽  
Shin-ichiro Watanabe ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Immunoglobulin Therapy ◽  
High Dose ◽  
Severe Bleeding ◽  
Bleeding Tendency ◽  
Intravenous Immunoglobulin Therapy ◽  
Acquired Von Willebrand Syndrome ◽  
Underlying Diseases ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract Of 260 patients enrolled, 25 patients (9.6%) were associated with acquired von Willebrand syndrome (AvWS). We studied 25 patients with AvWS, retrospectively. AvWS was diagnosed by reduced levels of von Willebrand factor (vWF) (decrease of von Willebrand factor antigen [vWF:Ag] and von Willebrand ristocetin cofactor [vWF:RCoF]), a decrease of ristocetin-induced platelet agglutination (RIPA), sometimes decreased high-molecular-weight multimers, and prolonged bleeding time with neither prior nor family histories of bleeding problems and the evidence of normal vWF:RCoF in their families. The inhibitor of vWF was determined by mixing patient plasma with pooled normal plasma. Eight patients in this study had the inhibitors to vWF that were of the IgG class; the subclasses were IgG1 (7 cases) and IgG2 (1 case). Multimeric analysis of vWF showed selective loss of large multimers in most patients with AvWS similar to that of congenital type-2 von Willebrand disease (vWD). All inhibitors blocked ristocetin-mediated vWF binding to platelets. Five out of 6 IgGs evaluated here recognized the 39/34-kD fragment (residues 480/481-718) and Fragment III (residues 1-1365) that implied binding domain of glycoprotein Ib (GPIb), whereas 1 recognized Fragment I (residues 911-1365). A close relationship was found between the presence of the inhibitor and bleeding tendency. Of the 7 patients with inhibitors, 6 patients (86%) had a bleeding tendency, as well as 1 of the 15 patients without inhibitors (6%). The efficacy of treatment of underlying diseases and/or therapy with deamino D-arginine vasopressin (DDAVP) for the treatment of AvWS also depends on the presence of an inhibitor. Four of 8 patients with inhibitors (50%) had poor response to treatment of the underlying disease and/or therapy with DDAVP, as well as 1 of the 16 patients without inhibitors (6%). These results indicate that patients with AvWS developing inhibitors to vWF are likely to have bleeding problems and might be resistant to treatment of underlying diseases and/or therapy with DDAVP for bleeding to AvWS. We also showed evidence that intravenous immunoglobulin therapy (0.3 g/kg, 3 days) was effective to correct a hemostatic defect and manage severe bleeding in a patient with AvWS developing inhibitors. We might consider an additional treatment including expensive high-dose immunoglobulin therapy when uncontrollable bleeding is continued after the treatment of the underlying diseases and/or therapy with DDAVP.

Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant

2016 ◽  
Vol 19 (3) ◽  
pp. 289-292 ◽  
Author(s):  
Ko Sakatsume ◽  
Masatoshi Akiyama ◽  
Kenki Saito ◽  
Shunsuke Kawamoto ◽  
Hisanori Horiuchi ◽  
...  
Keyword(s):  
Bleeding Tendency ◽  
Acquired Von Willebrand Syndrome ◽  
Jarvik 2000 ◽  
Von Willebrand

scholarly journals Utility of Various Von Willebrant Factor Laboratory Tests in Assessment of Acquired Von Willebrant Syndrome in Patients with Aortic Stenosis

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1517-1517
Author(s):  
Deepti M. Warad ◽  
Rachel Leger ◽  
Colleen S Thomas ◽  
Ewa M. Wysokinska ◽  
Matthew Auton ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Roc Curve ◽  
Conflicts Of Interest ◽  
Rank Correlation ◽  
Adenosine Diphosphate ◽  
Bleeding Tendency ◽  
Operating Characteristics ◽  
Acquired Von Willebrand Syndrome ◽  
Clinically Significant ◽  
Von Willebrand

Abstract Background: Aortic stenosis-associated acquired von Willebrand syndrome (AS-AVWS) is caused by an accelerated degradation of the highest molecular weight von Willebrand factor (VWF) multimers (HMWM). We recently reported that the severity of the HMWM loss correlates with the severity of AS and patients’ bleeding tendency (Am J Cardiol, 2013;111:374-81). As for the missing HMWM, it is still uncertain if they are cleared from circulation or redistributed with medium-low MWMs, which can be assessed by VWF propeptide (Pro)/antigen (Ag) ratio. Besides the tedious VWF multimer analysis, it is uncertain if VWF activity by immunoturbidic method (Lx)/ Ag ratio or VWF collagen binding activity (CBA)/Ag ratio can be used as a screening test to predict AS-AVWS. It has been speculated in the literature that VWF:CBA is more sensitive than VWF:Lx for detecting a HMWM loss. Our goal was to exam the laboratory characteristics of various VWF tests in evaluating AS-AVWS and the potential mechanism of HMWM loss. Method: Sixty-six patients (between years 2010-2012, 43% male) with varying degrees of AS (16 mild, 20 moderate, and 30 severe) and separate 21 patients who had aortic valve replacement were assessed with VWF:Ag, VWF:Lx, VWF:CBA, platelet function analyzer collagen plus adenosine diphosphate (PFA-CADP), VWF multimer analysis and multimer ratio by densitometry (Am J Cardiol, 2013;111:374-81) after their echocardiography. The clinical endpoints of this study are AS severity measured by mean gradient (MG) by echocardiography and clinically significant bleeding. Statistical analyses including Spearman rank correlation test, estimation of the area under the receiver operating characteristics (ROC) curve, and Wilcoxon rank sum tests were performed. Results: VWF:Lx and VWF:CBA had strong correlation (Spearman r=0.94, P<0.001), and the correlation of VWF:CBA/Ag and VWF:Ltx/Ag was 0.49, p<0.001). As previously published, in patients with AS, MG correlated with VWF:Lx/Ag (Spearman r = -0.41, p <0.001), PFA-CADP (r = 0.49, p <0.001), and VWF multimer ratio (r = -0.76, p <0.001). MG also correlated with VWF:CBA/Ag (r= -0.38, p=0.002), but not with VWF: Pro/Ag (r=-0.20, p=0.12). Among AS and AVR patients, the area under the ROC curve for detection of the loss of HMWM was 0.88 (95% CI: 0.80-0.95) by VWF multimer ratio, 0.76 (95% CI: 0.65-0.86) by PFA-CADP, 0.70 (95% CI: 0.58-0.82) by VWF:CBA/Ag ratio, 0.67 (95% CI: 0.56-0.70) by VWF:Lx/Ag ratio, and 0.53 (95% CI: 0.41-0.65) by VWF:Pro/Ag . In addition to previously published results, clinically significant bleeding, in 14% of AS patients, was not associated with VWF:CBA/Ag (p=0.25) or VWF:Pro/Ag ratio (p=0.83). VWF: CBA/Ag ratio was significantly lower in patients with severe AS (MG > 40 mmHg) compared to AVR patients (median: 0.84 vs. 1.06, p<0.001), while there was no evidence of a difference in VWF:Pro/Ag (p=0.45). Conclusion: HMWM losses are highly prevalent in patients with moderate to severe AS. Of the VWF tests, PFA-CADP and VWF multimer analysis and ratio had the highest predictive ability for AS-AVWS followed by VWF:CBA/Ag and VWF:Lx/Ag. The VWF:CBA is not overly superior than VWF:Lx for predicting a HMWM loss. The normal VWF:Pro/Ag suggests that AS-AVWS is different from other types of AVWS due to accelerated clearance of HMWM. Disclosures No relevant conflicts of interest to declare.

scholarly journals Clinical Significance of Inhibitors in Acquired von Willebrand Syndrome

Blood ◽  
1998 ◽  
Vol 91 (10) ◽  
pp. 3623-3629
Author(s):  
Hiroshi Mohri ◽  
Shigeki Motomura ◽  
Heiwa Kanamori ◽  
Michio Matsuzaki ◽  
Shin-ichiro Watanabe ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Immunoglobulin Therapy ◽  
High Dose ◽  
Severe Bleeding ◽  
Bleeding Tendency ◽  
Intravenous Immunoglobulin Therapy ◽  
Acquired Von Willebrand Syndrome ◽  
Underlying Diseases ◽  
Von Willebrand ◽  
Willebrand Factor

Of 260 patients enrolled, 25 patients (9.6%) were associated with acquired von Willebrand syndrome (AvWS). We studied 25 patients with AvWS, retrospectively. AvWS was diagnosed by reduced levels of von Willebrand factor (vWF) (decrease of von Willebrand factor antigen [vWF:Ag] and von Willebrand ristocetin cofactor [vWF:RCoF]), a decrease of ristocetin-induced platelet agglutination (RIPA), sometimes decreased high-molecular-weight multimers, and prolonged bleeding time with neither prior nor family histories of bleeding problems and the evidence of normal vWF:RCoF in their families. The inhibitor of vWF was determined by mixing patient plasma with pooled normal plasma. Eight patients in this study had the inhibitors to vWF that were of the IgG class; the subclasses were IgG1 (7 cases) and IgG2 (1 case). Multimeric analysis of vWF showed selective loss of large multimers in most patients with AvWS similar to that of congenital type-2 von Willebrand disease (vWD). All inhibitors blocked ristocetin-mediated vWF binding to platelets. Five out of 6 IgGs evaluated here recognized the 39/34-kD fragment (residues 480/481-718) and Fragment III (residues 1-1365) that implied binding domain of glycoprotein Ib (GPIb), whereas 1 recognized Fragment I (residues 911-1365). A close relationship was found between the presence of the inhibitor and bleeding tendency. Of the 7 patients with inhibitors, 6 patients (86%) had a bleeding tendency, as well as 1 of the 15 patients without inhibitors (6%). The efficacy of treatment of underlying diseases and/or therapy with deamino D-arginine vasopressin (DDAVP) for the treatment of AvWS also depends on the presence of an inhibitor. Four of 8 patients with inhibitors (50%) had poor response to treatment of the underlying disease and/or therapy with DDAVP, as well as 1 of the 16 patients without inhibitors (6%). These results indicate that patients with AvWS developing inhibitors to vWF are likely to have bleeding problems and might be resistant to treatment of underlying diseases and/or therapy with DDAVP for bleeding to AvWS. We also showed evidence that intravenous immunoglobulin therapy (0.3 g/kg, 3 days) was effective to correct a hemostatic defect and manage severe bleeding in a patient with AvWS developing inhibitors. We might consider an additional treatment including expensive high-dose immunoglobulin therapy when uncontrollable bleeding is continued after the treatment of the underlying diseases and/or therapy with DDAVP.

Von Willebrand Disease (vWd) Characterized by Increased Ristocetin Aggregation: A Study of Eleven Cases

1975 ◽  
Author(s):  
N. Ciavarella ◽  
F. I. Pareti ◽  
Z. M. Ruggeri ◽  
P. M. Mannucci
Keyword(s):  
Factor Viii ◽  
Bleeding Time ◽  
Autosomal Dominant ◽  
Von Willebrand Disease ◽  
Bleeding Tendency ◽  
Laboratory Findings ◽  
Prolonged Bleeding Time ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Antihemophilic Factor

The defective ristocetin aggregation occurring in patients with vWd is thought to depend on the decrease of a plasmatic factor related to factor VIII (Willebrand factor, VIIIVWF) 10 patients from 3 families had a mild to moderate bleeding tendency with autosomal dominant pattern of inheritance and laboratory findings suggestive for vWd (decreased levels of antihemophilic factor, and factor- VIII related antigen reduced platelet retention to glass beads columns and prolonged bleeding time). However, ristocetin aggregation in PRP was markedly increased, although VIIIVWF plasma levels were decreased. Aggregation induced in PRP by other agents (such as ADP, adrenaline and collagen), and the release of 14C serotonin was normal; the addition of purified bovine factor VIII was followed by normal aggregation in patients’ PRP and washed platelets. Patients’ washed platelets added to normal plasma aggregated to ristocetin more than normal washed platelets with patients’ plasma; the most marked aggregation response, however, was obtained when patients’ washed platelets were mixed with their own plasma. These findings suggest that a, platelet component is involved in the hyperaggregation response to ristocetin of these patients; however, the interaction of platelets with patients’ plasma is needed to produce the maximum response.Supported by a grant of the Fondazione Angelo Bianchi Bonomi.

Anti-Von Willebrand Factor Antibody Is Responsible for Lower Levels of Plasma vWF in Blood Group O Individuals.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 254-254 ◽  
Author(s):  
Letian Dai ◽  
Shawn Cotton ◽  
Alistair Macartney ◽  
Geoffrey Savidge ◽  
Anwar Alhaq
Keyword(s):  
Blood Group ◽  
Blood Groups ◽  
Bleeding Tendency ◽  
Plasma Samples ◽  
Abo Blood Groups ◽  
Acquired Von Willebrand Syndrome ◽  
Significant Difference ◽  
Group A ◽  
Group B ◽  
Von Willebrand

Abstract Plasma levels of vWF are known to be influenced by ABO blood groups, although the mechanism remains unresolved. Group O individuals have a significantly lower level of plasma vWF than those with group A, B or AB. This relative lower level of plasma vWF may result in a bleeding tendency and a shorter half-life of infused factor VIII in group O individuals. The formation of immune complexes between vWF and autoantibodies has been shown to accelerate vWF clearance from plasma in acquired von Willebrand syndrome. However, so far no evidence has been presented that the presence of autoantibodies against vWF is involved in lowering plasma level of vWF in group O individuals. In the present study, plasma samples were obtained from 199 healthy blood donors of blood group O (50), group A (50), group B (49), and group AB (50). A time-resolved fluorescence immunoassay (TRFIA) was developed to detect anti-vWF IgG in plasma samples. Briefly, 100 μl of diluted plasma was loaded on to duplicated vWF-coated and untreated control wells of a microplate. After incubation and washing, 100 μl of Europium-labeled anti-human IgG conjugate (1:500 dilution) was added to the plate to detect vWF IgG. The time-delayed fluorescence was then measured with a Victor microplate reader (PerkinElmer, Turku, Finland). The fluorescence counts of the control wells were subtracted from those of the vWF-coated wells. The results show that anti-vWF IgG was present in all four blood groups (Table 1). Of these blood groups, group O had the highest anti-vWF IgG level with 9.8 x 105 fluorescence counts, which was 2.7- to 3.5-fold higher than that of group A, B or AB. There was a significant difference in the anti-vWF IgG levels between group O and the rest of group A, B or AB. Quantitative analysis of plasma vWF by ELISA showed that the concentration of plasma vWF of group O was 29 to 35% lower than that of group A, B or AB (Table 1). These results suggest that TRFIA is a sensitive assay for detection of anti-vWF IgG in plasma samples, and the presence of the high level of anti-vWF Ig G in group O individuals may be responsible for lowing plasma vWF by acceleration of vWF clearance. Anti-VWF IgG levels and vWF concentrations in diffeent ABO blood groups Group O (n=50) Group A (n=50) Group B (n=49) Group AB (n=50) Data are presented as a mean ± SD. *P<0.01 compared with group A, B or AB. +P<0.01 compared with group A,B or AB. Anti-vWF IgG (x 105 Fluorescence counts) 9.8 ± 6.9* 2.8 ± 2.8 3.1 ± 3.0 3.6 ± 3.5 Concentration of vWF (% of normal controls) 116 ± 42+ 165 ± 46 165 ± 42 179 ± 49

Interactions of von Willebrand factor and ADAMTS13 in von Willebrand disease and thrombotic thrombocytopenic purpura

2014 ◽  
Vol 34 (03) ◽  
pp. 215-225 ◽  
Author(s):  
R. Schneppenheim ◽  
U. Budde
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Von Willebrand Factor ◽  
Active Phase ◽  
Von Willebrand Disease ◽  
Bleeding Tendency ◽  
Thrombocytopenic Purpura ◽  
Acquired Von Willebrand Syndrome ◽  
Key Factor ◽  
Von Willebrand ◽  
Willebrand Factor

SummaryThe function of von Willebrand factor (VWF), a huge multimeric protein and a key factor in platelet dependent primary haemostasis, is regulated by its specific protease ADAMTS13. The ADAMTS13 dependent degradation of VWF to its proteolytic fragments can be visualized as a characteristic so-called triplet structure of individual VWF oligomers by multimer analysis. Lack of VWF high molecular weight multimers (VWF-HMWM) or their pathologically enhanced degradation under - lies a particular type of von Willebrand disease, VWD type 2A with a significant bleeding tendency, and may also be observed in acquired von Willebrand syndrome due to cardiovascular disease. In these conditions multimer analysis is an obligatory and powerful tool for diagnosis of VWD. The opposite condition, the persistence of ultralarge VWF (UL-VWF) multimers may cause the microangiopathic life-threatening disorder thrombotic thrombocytopenic purpura (TTP). During the course of active TTP, UL-VWF is consumed in the hyaline thrombi formed in the microvasculature which will ultimately result in the loss of UL-VWF and VWF-HMWM. Therefore, VWF multimer analysis is not a valid tool to diagnose TTP in the active phase of disease but may be helpful for the diagnosis of TTP patients in remission.

scholarly journals Role of acquired von Willebrand syndrome in the development of bleeding complications in patients treated with Impella RP devices

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Mehmet Oezkur ◽  
Sara Reda ◽  
Heiko Rühl ◽  
Nils Theuerkauf ◽  
Stefan Kreyer ◽  
...  
Keyword(s):  
Academic Research ◽  
Axial Flow ◽  
Left Ventricular ◽  
Bleeding Complications ◽  
Laboratory Findings ◽  
Acquired Von Willebrand Syndrome ◽  
Intravenous Heparin ◽  
Ventricular Support ◽  
Research Consortium ◽  
Von Willebrand

AbstractAxial flow pumps are standard treatment in cases of cardiogenic shock and high-risk interventions in cardiology and cardiac surgery, although the optimal anticoagulation strategy remains unclear. We evaluated whether laboratory findings could predict bleeding complications and acquired von Willebrand syndrome (avWS) among patients who were treated using axial flow pumps. We retrospectively evaluated 60 consecutive patients who received Impella devices (Impella RP: n = 20, Impella CP/5.0: n = 40; Abiomed Inc., Danvers, USA) between January 2019 and December 2020. Thirty-two patients (53.3%) experienced major or fatal bleeding complications (Bleeding Academic Research Consortium score of > 3) despite intravenous heparin being used to maintain normal activated partial thromboplastin times (40–50 s). Extensive testing was performed for 28 patients with bleeding complications (87.5%). Relative to patients with left ventricular support, patients with right ventricular support were less likely to develop avWS (87.5% vs. 58.8%, p = 0.035). Bleeding was significantly associated with avWS (odds ratio [OR]: 20.8, 95% confidence interval [CI]: 3.3–128.5; p = 0.001) and treatment duration (OR: 1.3, 95% CI 1.09–1.55; p = 0.003). Patients with avWS had longer Impella treatment than patients without avWS (2 days [1–4.7 days] vs. 7.3 days [3.2–13.0 days]). Bleeding complications during Impella support were associated with avWS in our cohort, while aPTT monitoring was not sufficient to prevent bleeding complications. A more targeted anticoagulation monitoring might be needed for patients who receive Impella devices.

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