Low plasma protein Z levels are associated with an increased risk for perioperative bleedings

Hinterleitner, Clemens; Kreißelmeier, Klaus-Peter; Pecher, Ann-Christin; Mauz, Paul-Stefan; Kanz, Lothar; Kopp, Hans‐Georg; Jaschonek, K.

doi:10.1111/ejh.13031

Cited by 5 publications

(3 citation statements)

References 34 publications

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“…Analyses of von Willebrand parameters (VWF:Ag, VWF:CB, VWF:RCo) and factor VIII activity (FVIII:C) were performed as described previously . Plasma samples for multimer analysis were stored frozen at −74 °C.…”

Section: Methodsmentioning

confidence: 99%

Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case‐series

Icheva

Nowak-Machen²,

Budde

et al. 2018

Journal of Thrombosis and Haemostasis

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Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well. SUMMARY: Background Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications. CHD-related anatomic features such as valve stenoses or patent arterial ducts can lead to enhanced shear stress in the blood stream and thus cause acquired von Willebrand syndrome (aVWS). Objective To evaluate the intraoperative incidence and impact of aVWS after cardiopulmonary bypass (CPB) in neonates and infants with complex CHD. Patients/Methods We conducted a survey of patients aged < 12 months undergoing complex cardiac surgery in our tertiary referral center. Twelve patients, whose blood samples were analyzed for aVWS before CPB and immediately after discontinuation of CPB on a routine basis, were eligible for the analysis. von Willebrand factor antigen (VWF:Ag), ristocetin cofactor activity (VWF:RCo), collagen binding activity (VWF:CB), VWF:multimers and factor VIII activity (FVIII:C) were determined. Results aVWS was diagnosed by VWF multimer analysis in 10 out of 12 patients (83%) prior to surgery and intraoperatively at the end of CPB in 8 out of 12 patients (66%). Ten patients received VWF/FVIII concentrate intraoperatively as individual treatment attempts during uncontrolled bleeding. They tolerated it well without intraoperative thrombotic events. One patient suffered a transient postoperative cerebral sinuous vein thrombosis. Conclusions aVWS is of underestimated incidence in complex CHD surgery. These data may offer a new approach to reduce the risk of severe bleedings and to achieve hemostasis during high-risk pediatric cardiac surgery by tailoring the substitution with von Willebrand factor concentrate.

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Section: Methodsmentioning

confidence: 99%

Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case‐series

Icheva

Nowak-Machen²,

Budde

et al. 2018

Journal of Thrombosis and Haemostasis

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“…Von Willebrand antigen (VWF:Ag), collagen binding (VWF:CB) and Willebrand multimers were analyzed in an external reference lab (cMEDILYS Coagulation Lab mbH, Hamburg, Germany) as described previously ( 19 , 20 ). The aVWS was graded as severe (++) if either a complete loss of the largest multimers was present or if the high molecular multimers were less than 20% of all multimers.…”

Section: Methodsmentioning

confidence: 99%

Acquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: Retrospective case-series

Icheva

Budde²,

Magunia

et al. 2022

Front. Pediatr.

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BackgroundAlthough acquired von Willebrand syndrome (aVWS) has been described in congenital heart disease before, anatomical features leading to aVWS with characteristic reduction or loss of high molecular weight von Willebrand multimers (HMWM) are not well known. This study assesses the prevalence and effects of aVWS in infants with systemic-to-pulmonary shunts (SPS).MethodsThis retrospective single-center study analyzes diagnostic data of infants with complex congenital heart defects requiring palliation with SPS. During the study period between 12/15–01/17 fifteen consecutive patients were eligible for analysis. Results of von Willebrand factor antigen (VWF:Ag), collagen binding activity (VWF:CB) and von Willebrand factor multimer analysis were included.ResultsIn all 15 patients with SPS an aVWS could be found. Blood samples were collected between 5 and 257 days after shunt implantation (median 64 days). None of the patients demonstrated increased bleeding in everyday life. However, 6 out of 15 patients (40%) showed postoperative bleeding complications after SPS implantation. Following shunt excision multimeric pattern normalized in 8 of 10 (80%) patients studied.ConclusionsThis study shows that in patients undergoing SPS implantation aVWS might emerge. Pathogenesis can be explained by shear stress resulting from turbulent flow within the shunt. Knowledge of aVWS existence is important for the consideration of replacement therapy with von Willebrand factor containing products and antifibrinolytic treatment in bleeding situations. Implementation of methods for rapid aVWS detection is required to achieve differentiated hemostatic therapy and reduce the risk of complications caused by empiric replacement therapy.

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“…VWF plasma multimers were analyzed using SDS‐agaroses gel electrophoresis. All methods used here have been described in detail previously …”

Section: Methodsmentioning

confidence: 99%

Disease progression and defects in primary hemostasis as major cause of bleeding in multiple myeloma

Hinterleitner

Pecher

Kreißelmeier

et al. 2019

European J of Haematology

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Background and objectives In patients with multiple myeloma (MM), unexpected bleeding complications remain a major issue. Since routine coagulation parameters are often inconspicuous, diagnosis and treatment of the underlying coagulation disorders are challenging. Patients and methods In our single‐center observational study, we analyzed 164 patients with MM for coagulation disorders and bleeding complications. Results Prolonged closure times (CTs), measured by PFA‐100, were the most common, abnormal coagulation test, found in 66% of bleeding patients vs 5% in non‐bleeding, followed by qualitative defects of von Willebrand factor (VWF:CB/VWF:Ag ratios), found in 34% vs 1% in the non‐bleeding group. Increased serum free light chains (SFLC) and SFLC ratios were significantly associated with prolonged CTs and acquired von Willebrand syndrome (AVWS). Prolonged CTs and AVWS were associated with disease progression, determined by dynamics of SFLC ratios (P < .001), serum creatinine level (P = .013), Beta‐2 microglobulin (P = .03), LDH (P = .016), and bone marrow infiltration (P < .001). Of note, response to myeloma therapy was frequently correlated with normalization of coagulation parameters. Conclusions Bleeding complications in MM are predominantly caused by defects in primary hemostasis and associated with disease progression. In a peri‐interventional workup, determination of CTs and VWF:CB/VWF:Ag ratios are of significant importance to assess bleeding risk.

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Low plasma protein Z levels are associated with an increased risk for perioperative bleedings

Abstract: Low PZ appears to be an underestimated risk factor for perioperative bleeding. Determination of PZ plasma concentrations might be useful in the preoperative workup in patients with a bleeding history, when detailed clotting analyses remain inconclusive.

Cited by 5 publications

References 34 publications

Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case‐series

Acquired von Willebrand syndrome in congenital heart disease surgery: results from an observational case‐series

Acquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: Retrospective case-series

Disease progression and defects in primary hemostasis as major cause of bleeding in multiple myeloma

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