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Case Reports
. 2023 Jun 21;23(1):217.
doi: 10.1186/s12871-023-02147-4.

Heparin-resistance in AL amyloidosis: a case report

Elisabeth S van Ede  1 , Marlijn P A Hoeks  2 , Jan Hofland  3 , Vera D Linssen  4 , Toin H van Kuppevelt  5 , Elly M Versteeg  5 , Theo G Hafmans  5 , Arjan Diepstra  6 , Benno Kusters  7 , Sanne M M Vermorgen  7
Affiliations
Case Reports

Heparin-resistance in AL amyloidosis: a case report

Elisabeth S van Ede et al. BMC Anesthesiol. .

Abstract

Background: Non-AT-III mediated heparin-resistance during CPB occurs by complex-forming with heparin-binding proteins. Currently, there are no specific recommendations for non-AT-III mediated heparin-resistance.

Case presentation: We present a fatal case of a 70-yr-old male-patient undergoing cardiac-surgery in which refractory heparin-resistance was observed. The massive AL amyloidosis found at autopsy is thought to be responsible and illustrates that awareness and knowledge of the etiology and perioperative strategies of non-AT-III mediated heparin-resistance is important.

Conclusion: For anticoagulation during cardiopulmonary bypass surgery in case of a non-AT-III medicated heparin resistance, we refer to the decision tree added to this manuscript and if necessary to consider direct thrombin inhibitors, such as bivalirudin or argatroban, as it bypasses the complexing pathway.

Keywords: AL amyloidosis; Anticoagulation; Case report; Heparin Resistance; Perfusion.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Amyloid Light-chain amyloid in liver and spleen. Macroscopic liver (upper left) and spleen (upper right). Hematoxylin–eosin stain technique liver (lower left; dark-purple spots = AL amyloid) and electron microscopy image spleen (lower right; black fibrils = AL amyloid)
Fig. 2
Fig. 2
Decision tree peroperative use anticoagulation during cardiopulmonary bypass surgery
See this image and copyright information in PMC

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