Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)

Abstract Background Cushing’s syndrome (CS) is associated with an hypercoagulable state and an increased risk of venous thromboembolism (VTE). Evidence-based guidelines on thromboprophylaxis strategies in patients with CS are currently lacking. We aimed to map the current clinical practice for throm...

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Main Authors: F. M. van Haalen, M. Kaya, I. C. M. Pelsma, O. M. Dekkers, N. R. Biermasz, S. C. Cannegieter, M. V. Huisman, B. J. M. van Vlijmen, R. A. Feelders, F. A. Klok, A. M. Pereira, Endo-ERN Cushing and Thrombosis study group
Format: Article
Language:English
Published: BMC 2022-05-01
Series:Orphanet Journal of Rare Diseases
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Online Access:https://doi.org/10.1186/s13023-022-02320-x
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author F. M. van Haalen
M. Kaya
I. C. M. Pelsma
O. M. Dekkers
N. R. Biermasz
S. C. Cannegieter
M. V. Huisman
B. J. M. van Vlijmen
R. A. Feelders
F. A. Klok
A. M. Pereira
Endo-ERN Cushing and Thrombosis study group
author_facet F. M. van Haalen
M. Kaya
I. C. M. Pelsma
O. M. Dekkers
N. R. Biermasz
S. C. Cannegieter
M. V. Huisman
B. J. M. van Vlijmen
R. A. Feelders
F. A. Klok
A. M. Pereira
Endo-ERN Cushing and Thrombosis study group
author_sort F. M. van Haalen
collection DOAJ
description Abstract Background Cushing’s syndrome (CS) is associated with an hypercoagulable state and an increased risk of venous thromboembolism (VTE). Evidence-based guidelines on thromboprophylaxis strategies in patients with CS are currently lacking. We aimed to map the current clinical practice for thromboprophylaxis management in patients with CS across reference centers (RCs) of the European Reference Network on Rare Endocrine Conditions (Endo-ERN), which are endorsed specifically for the diagnosis and treatment of CS. Using the EU survey tool, a primary screening survey, and subsequently a secondary, more in-depth survey were developed. Results The majority of the RCs provided thromboprophylaxis to patients with CS (n = 23/25), although only one center had a standardized thromboprophylaxis protocol (n = 1/23). RCs most frequently started thromboprophylaxis from CS diagnosis onwards (n = 11/23), and the majority stopped thromboprophylaxis based on individual patient characteristics, rather than standardized treatment duration (n = 15/23). Factors influencing the initiation of thromboprophylaxis were ‘medical history of VTE’ (n = 15/23) and ‘severity of hypercortisolism’ (n = 15/23). Low-Molecular-Weight-Heparin was selected as the first-choice anticoagulant drug for thromboprophylaxis by all RCs (n = 23/23). Postoperatively, the majority of RCs reported ‘severe immobilization’ as an indication to start thromboprophylaxis in patients with CS (n = 15/25). Most RCs (n = 19/25) did not provide standardized testing for variables of hemostasis in the postoperative care of CS. Furthermore, the majority of the RCs provided preoperative medical treatment to patients with CS (n = 23/25). About half of these RCs (n = 12/23) took a previous VTE into account when starting preoperative medical treatment, and about two-thirds (n = 15/23) included ‘reduction of VTE risk’ as a goal of treatment. Conclusions There is a large practice variation regarding thromboprophylaxis management and perioperative medical treatment in patients with CS, even in Endo-ERN RCs. Randomized controlled trials are needed to establish the optimal prophylactic anticoagulant regimen, carefully balancing the increased risk of (perioperative) bleeding, and the presence of additional risk factors for thrombosis.
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spelling doaj.art-2cca4a39f38c4dd2b220a26962101ae92022-12-22T00:45:28ZengBMCOrphanet Journal of Rare Diseases1750-11722022-05-0117111210.1186/s13023-022-02320-xCurrent clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)F. M. van Haalen0M. Kaya1I. C. M. Pelsma2O. M. Dekkers3N. R. Biermasz4S. C. Cannegieter5M. V. Huisman6B. J. M. van Vlijmen7R. A. Feelders8F. A. Klok9A. M. Pereira10Endo-ERN Cushing and Thrombosis study groupDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterDivision of Thrombosis and Hemostasis, Leiden University Medical CenterDivision of Thrombosis and Hemostasis, Leiden University Medical CenterDivision of Thrombosis and Hemostasis, Leiden University Medical CenterDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterDivision of Thrombosis and Hemostasis, Leiden University Medical CenterDepartment of Medicine, Division of Endocrinology, Leiden University Medical CenterAbstract Background Cushing’s syndrome (CS) is associated with an hypercoagulable state and an increased risk of venous thromboembolism (VTE). Evidence-based guidelines on thromboprophylaxis strategies in patients with CS are currently lacking. We aimed to map the current clinical practice for thromboprophylaxis management in patients with CS across reference centers (RCs) of the European Reference Network on Rare Endocrine Conditions (Endo-ERN), which are endorsed specifically for the diagnosis and treatment of CS. Using the EU survey tool, a primary screening survey, and subsequently a secondary, more in-depth survey were developed. Results The majority of the RCs provided thromboprophylaxis to patients with CS (n = 23/25), although only one center had a standardized thromboprophylaxis protocol (n = 1/23). RCs most frequently started thromboprophylaxis from CS diagnosis onwards (n = 11/23), and the majority stopped thromboprophylaxis based on individual patient characteristics, rather than standardized treatment duration (n = 15/23). Factors influencing the initiation of thromboprophylaxis were ‘medical history of VTE’ (n = 15/23) and ‘severity of hypercortisolism’ (n = 15/23). Low-Molecular-Weight-Heparin was selected as the first-choice anticoagulant drug for thromboprophylaxis by all RCs (n = 23/23). Postoperatively, the majority of RCs reported ‘severe immobilization’ as an indication to start thromboprophylaxis in patients with CS (n = 15/25). Most RCs (n = 19/25) did not provide standardized testing for variables of hemostasis in the postoperative care of CS. Furthermore, the majority of the RCs provided preoperative medical treatment to patients with CS (n = 23/25). About half of these RCs (n = 12/23) took a previous VTE into account when starting preoperative medical treatment, and about two-thirds (n = 15/23) included ‘reduction of VTE risk’ as a goal of treatment. Conclusions There is a large practice variation regarding thromboprophylaxis management and perioperative medical treatment in patients with CS, even in Endo-ERN RCs. Randomized controlled trials are needed to establish the optimal prophylactic anticoagulant regimen, carefully balancing the increased risk of (perioperative) bleeding, and the presence of additional risk factors for thrombosis.https://doi.org/10.1186/s13023-022-02320-xCushing’s syndromeHypercortisolismHemostasisVenous thromboembolismThromboprophylaxisGuidelines
spellingShingle F. M. van Haalen
M. Kaya
I. C. M. Pelsma
O. M. Dekkers
N. R. Biermasz
S. C. Cannegieter
M. V. Huisman
B. J. M. van Vlijmen
R. A. Feelders
F. A. Klok
A. M. Pereira
Endo-ERN Cushing and Thrombosis study group
Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)
Orphanet Journal of Rare Diseases
Cushing’s syndrome
Hypercortisolism
Hemostasis
Venous thromboembolism
Thromboprophylaxis
Guidelines
title Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)
title_full Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)
title_fullStr Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)
title_full_unstemmed Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)
title_short Current clinical practice for thromboprophylaxis management in patients with Cushing’s syndrome across reference centers of the European Reference Network on Rare Endocrine Conditions (Endo-ERN)
title_sort current clinical practice for thromboprophylaxis management in patients with cushing s syndrome across reference centers of the european reference network on rare endocrine conditions endo ern
topic Cushing’s syndrome
Hypercortisolism
Hemostasis
Venous thromboembolism
Thromboprophylaxis
Guidelines
url https://doi.org/10.1186/s13023-022-02320-x
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