scholarly journals Acquired von Willebrand Syndrome Complicating Nephrotic Syndrome: A Case of a Patient With Membranous Nephropathy

2018 ◽  
Vol 11 ◽  
pp. 117954761876337
Author(s):  
Taro Sugase ◽  
Tetsu Akimoto ◽  
Takaaki Kimura ◽  
Takashi Yagisawa ◽  
Eiji Kusano ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Major Trauma ◽  
Invasive Procedure ◽  
Bleeding Risk ◽  
Current Case ◽  
Acquired Von Willebrand Syndrome ◽  
Active Partial Thromboplastin Time ◽  
Von Willebrand ◽  
Prompt Diagnosis

Acquired von Willebrand syndrome (AVWS) is a rare clinical entity presenting with heterogeneous hemorrhagic manifestations, although some subsets of patients with AVWS may be asymptomatic until they are exposed to major trauma, an invasive procedure, or surgery. We herein report one such case in a 73-year-old male patient with nephrotic syndrome with a prolonged active partial thromboplastin time. We initially did not deal with this distinct abnormal clotting profile seriously, but persistent bleeding after a retroperitoneoscopic-assisted renal biopsy that allowed us to ascribe his nephrotic syndrome to membranous nephropathy fortuitously led to the discovery of concurrent AVWS. We feel that an accurate and prompt diagnosis as well as awareness of the disease remain a challenge for physicians and therefore strongly recommend the further accumulation of experiences similar to our own in a prospective manner. This report underscores the pitfalls associated with determining the bleeding risk, including an insufficient assessment and improper weighting of an abnormal clotting profile prior to the invasive procedure. Several management concerns that emerged in the current case are also discussed.

scholarly journals Essential Thrombocythemia and Acquired von Willebrand Syndrome: The Shadowlands between Thrombosis and Bleeding

Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1746 ◽  
Author(s):  
Hassan Awada ◽  
Maria Voso ◽  
Paola Guglielmelli ◽  
Carmelo Gurnari
Keyword(s):  
Essential Thrombocythemia ◽  
Clinical Evidence ◽  
Antiplatelet Agents ◽  
Bleeding Risk ◽  
Clinical Approach ◽  
Evidence Based ◽  
Vascular Events ◽  
Acquired Von Willebrand Syndrome ◽  
The Past ◽  
Von Willebrand

Over the past decade, new insights have emerged on the pathophysiology of essential thrombocythemia (ET), its clinical management, and associated thrombohemostatic disturbances. Here, we review the latest diagnostic and risk stratification modalities of ET and its therapeutics. Moreover, we discuss the clinical evidence-based benefits, deriving from major clinical trials, of using cytoreductive therapy and antiplatelet agents to lower the risk of fatal vascular events. Also, we focus on the condition of extreme thrombocytosis (>1000 × 109/L) and bleeding risk, the development and pathogenesis of acquired von Willebrand syndrome, and the clinical approach to this paradoxical scenario in ET.

scholarly journals Acquired von Willebrand Syndrome and Platelet Function Defects during Extracorporeal Life Support (Mechanical Circulatory Support)

2020 ◽  
Vol 40 (02) ◽  
pp. 221-225
Author(s):  
Axel Schlagenhauf ◽  
Johannes Kalbhenn ◽  
Ulrich Geisen ◽  
Friedhelm Beyersdorf ◽  
Barbara Zieger
Keyword(s):  
Shear Stress ◽  
Life Support ◽  
Bleeding Risk ◽  
Ventricular Assist Devices ◽  
Circulatory Support ◽  
Recurrent Bleeding ◽  
Mechanical Destruction ◽  
Acquired Von Willebrand Syndrome ◽  
Increased Risk ◽  
Von Willebrand

AbstractPatients with ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO) suffer from an increased risk for thromboembolic events as well as for hemorrhages. High shear stress in the mechanical device results in acquired von Willebrand syndrome (AVWS), characterized by a loss of high-molecular-weight multimers of von Willebrand factor (VWF) leading to an increased bleeding risk. Onset of AVWS occurs within hours, persists during the whole period of mechanical support, and subsides rapidly after explantation. Patients with the older HeartMate II exhibit more severe AVWS than those with the newer HeartMate III, thanks to lower shear stress in the latter. All ECMO and VAD patients exhibit thrombocytopathia and often thrombocytopenia which further increases the bleeding risk. Etiological models for AVWS are increased cleavage by the metalloproteinase ADAMSTS13, mechanical destruction of VWF, and shear-induced VWF binding to platelets. Platelet secretion defects may be caused by transient platelet activation leading to degranulation. AVWS can be diagnosed by detection of VWF multimers using gel-electrophoresis and functional assays of varying sensitivity (VWF ristocetin cofactor activity, VWF activity, VWF collagen binding). Platelet dysfunction is monitored using light transmission aggregometry and secretion defects are detectable using flow cytometry. Modest use of anticoagulants and a target-controlled therapy based on VWF parameters and other coagulation and platelet parameters are shown to be beneficial in this patient group. Persistent hemorrhages may be controlled with tranexamic acid and platelet concentrates. Prompt weaning from the device, when indicated, is the best therapeutic option to prevent recurrent bleeding.

scholarly journals Optimal tests to minimise bleeding and ischaemic complications in patients on short-term mechanical circulatory support

2021 ◽  
Author(s):  
Rahim Kanji ◽  
Christophe Vandenbriele ◽  
Deepa Arachchillage ◽  
Susanna Price ◽  
Diana Adrienne Gorog
Keyword(s):  
Platelet Aggregation ◽  
Platelet Function ◽  
Mechanical Circulatory Support ◽  
Factor Xa ◽  
Bleeding Risk ◽  
Circulatory Support ◽  
Optimal Test ◽  
Very High Frequency ◽  
Acquired Von Willebrand Syndrome ◽  
Von Willebrand

Patients receiving left-sided mechanical circulatory support (MCS) require systemic anticoagulation with unfractionated heparin (UFH) to prevent clotting of the circuit and reduce the risk of arterial thrombosis. With MCS, there is a very high frequency of bleeding and ischaemic complications, including stroke and systemic embolism. Monitoring of UFH can be very challenging. Whilst most centres routinely monitor the activated clotting time, prothrombin time, activated partial thromboplastin time (aPTT) and fibrinogen to assess haemostasis, there is no clear guidance available regarding the optimal test. Additional tests, including antithrombin level, anti-factor Xa assay and thromboelastography can be used for risk stratification of patients to try and predict the risks of thrombosis and bleeding. Each has their specific role, strengths and limitations. Measurement of anti-Xa level best correlates with heparin dose, and appears predictive of circuit thrombosis, although aPTT is a better predictor of bleeding. Increased thrombin generation may have a role in predicting thrombosis. Acquired von Willebrand syndrome is frequent with MCS, contributing to bleeding risk and can be detected by assessing the von Willebrand factor activity to antigen ratio, whilst the Platelet Function Analyzer can be used in urgent situations with high negative predictive value. Tests of platelet aggregation can aid the prediction bleeding. A selection of complementary tests to collectively assess heparin-effect, coagulation, platelet function and platelet aggregation is recommended to personalise management and thereby optimise outcomes in patients receiving MCS.

scholarly journals Acquired von Willebrand Syndrome in left Impella supported cardiogenic shock patients

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Vandenbriele ◽  
T Balthazar ◽  
M Engelen ◽  
T Adriaenssens ◽  
P Verhamme ◽  
...  
Keyword(s):  
Cardiogenic Shock ◽  
Critically Ill ◽  
Bleeding Risk ◽  
Rapid Onset ◽  
Ventricular Assist Devices ◽  
Circulatory Support ◽  
Paired Data ◽  
Acquired Von Willebrand Syndrome ◽  
Off Pump ◽  
Von Willebrand

Abstract Background Bleeding is a main cause of morbidity and mortality in critically ill cardiogenic shock patients, supported by short-term percutaneous mechanical circulatory support (pMCS) devices. Bleeding not only occurs because of obligatory heparin and antiplatelet therapy (both required in the prevention of pump and stent thrombosis) but possibly also results from device-related coagulopathy. Similar to long-term ventricular assist devices, mechanical shear-induced acquired von Willebrand syndrome (AVWS) might further increase the bleeding risk. Therefore, we aimed to investigate the effect of left Impella percutaneous continuous flow pumps on the development of AVWS due to shear-induced excessive cleavage of large vWF multimers by the metalloproteinase ADAMTS-13, resulting in loss of high-molecular-weight vWF multimers. Methods Between March 2019 and January 2020, all cardiogenic shock patients supported by a left Impella and referred to a single tertiary ICU were studied. Both vWF Antigen (vWF:Ag) and vWF:GPIbR (ristocetin-induced binding of vWF to a recombinant wildtype Glycoprotein Ib fragment) levels were measured by chemiluminescent immunoassays using an AcuStar (Werfen) assay to determine the vWF:GPIbR /vWF:Ag ratio (normal range ≥1.0). VWF multimer analysis was performed by electrophoresis. On-pump analyses were performed 12h after implantation and off-pump analyses 12h after Impella explantation. Patients who died on-pump were excluded because of lack of paired data after explantation. Results Eight left Impella patients (four Impella CP, four Impella 5.0) were analyzed for AVWS. The vWF:GPIbR /vWF:Ag ratio was <1.0 in all patients on-pump (mean±SD 0.68±0.1 versus 1.1±0.15 off-pump (panel A; p=0.0018)) and thus AVWS was detected in all Impella-supported patients. The presence of AVSW was also confirmed by loss of large vWF multimers on-pump (panel B). Four patients (50%) had mucosal bleeds (epistaxis or gastrointestinal), none of them requiring transfusion. The mean rise in ratio 12h after pump removal was 0.35 which was also reflected by recovery of large multimers by electrophoresis (panel B). Conclusions Our data highlight the rapid onset and reversal of AVWS in all studied cardiogenic shock patients, supported by a left Impella pump. The determination of the GPIbR /vWF:Ag ratio with the AcuStar appears a reliable and faster test to detect AVWS as compared to vWF multimers electrophoresis. Further research into innovative pharmacological interventions (e.g. ADAMTS-13 inhibitors) should target pMCS-induced AVWS in an effort to reduce hemostatic complications in this critically ill ICU population. AVWS in Impella supported patients Funding Acknowledgement Type of funding source: None

Perioperative Management of DOACs in Vascular Surgery: A Practical Approach

2019 ◽  
Vol 24 (38) ◽  
pp. 4518-4524 ◽  
Author(s):  
George Kouvelos ◽  
Miltiadis Matsagkas ◽  
Nikolaos Rousas ◽  
Petroula Nana ◽  
Konstantinos Mpatzalexis ◽  
...  
Keyword(s):  
Vascular Surgery ◽  
Perioperative Management ◽  
Invasive Procedure ◽  
Bleeding Risk ◽  
Low Risk ◽  
Practical Approach ◽  
Current Evidence ◽  
Thromboembolic Risk ◽  
Specific Data ◽  
Vascular Procedure

Background: Approximately 10–15% of patients on DOACs have to interrupt their anticoagulant before an invasive procedure every year. The perioperative management and monitoring of DOACs have proved to be challenging, as differences in patients’ status and in the invasiveness of each procedure develop different situations that need a tailored therapeutic approach to each patient’s needs. Methods: This review aims to summarize current evidence on the perioperative management of DOACs in patients undergoing a vascular surgical procedure focusing with a practical approach on three key clinical questions: (i) can we stop DOAC therapy before the vascular procedure? (ii) is bridging therapy necessary? and (iii) which is the best perioperative strategy for interruption and resumption of the anticoagulant therapy? Results: No specific data exist for the perioperative management of vascular surgery patients on DOACs, as most studies include low number of such patients. Therapeutic strategy on how to handle DOACs perioperatively must be based on their half-life, the bleeding risk of the invasive procedures, and on the thromboembolic risk of the patient. Renal function plays a crucial role in such situations, increasing thromboembolic and bleeding risk. In general, DOACs should be stopped 2 days for high bleed risk, 1 day for low risk and should be resumed 48-72 hrs after high risk, 24 hrs after low-risk procedure. Bridging is almost never needed. Conclusion: Further perioperative research studies on patients undergoing vascular surgery are needed to confirm whether currently accepted therapeutic perioperative strategy is appropriate for these patients.

Toward Personalized Treatment for Patients with Low von Willebrand Factor and Quantitative von Willebrand Disease

2021 ◽  
Vol 47 (02) ◽  
pp. 192-200
Author(s):  
James S. O'Donnell
Keyword(s):  
Von Willebrand Factor ◽  
Bleeding Risk ◽  
Von Willebrand Disease ◽  
Personalized Treatment ◽  
Treatment Plans ◽  
Type 3 ◽  
Von Willebrand ◽  
Willebrand Factor

AbstractThe biological mechanisms involved in the pathogenesis of type 2 and type 3 von Willebrand disease (VWD) have been studied extensively. In contrast, although accounting for the majority of VWD cases, the pathobiology underlying partial quantitative VWD has remained somewhat elusive. However, important insights have been attained following several recent cohort studies that have investigated mechanisms in patients with type 1 VWD and low von Willebrand factor (VWF), respectively. These studies have demonstrated that reduced plasma VWF levels may result from either (1) decreased VWF biosynthesis and/or secretion in endothelial cells and (2) pathological increased VWF clearance. In addition, it has become clear that some patients with only mild to moderate reductions in plasma VWF levels in the 30 to 50 IU/dL range may have significant bleeding phenotypes. Importantly in these low VWF patients, bleeding risk fails to correlate with plasma VWF levels and inheritance is typically independent of the VWF gene. Although plasma VWF levels may increase to > 50 IU/dL with progressive aging or pregnancy in these subjects, emerging data suggest that this apparent normalization in VWF levels does not necessarily equate to a complete correction in bleeding phenotype in patients with partial quantitative VWD. In this review, these recent advances in our understanding of quantitative VWD pathogenesis are discussed. Furthermore, the translational implications of these emerging findings are considered, particularly with respect to designing personalized treatment plans for VWD patients undergoing elective procedures.

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