Heparin-resistance in AL amyloidosis: a case report

Elisabeth S van Ede; Marlijn P A Hoeks; Jan Hofland; Vera D Linssen; Toin H van Kuppevelt; Elly M Versteeg; Theo G Hafmans; Arjan Diepstra; Benno Kusters; Sanne M M Vermorgen

doi:10.1186/s12871-023-02147-4

Heparin-resistance in AL amyloidosis: a case report

Elisabeth S van Ede, Marlijn P A Hoeks, Jan Hofland, Vera D Linssen^*, Toin H van Kuppevelt, Elly M Versteeg, Theo G Hafmans, Arjan Diepstra, Benno Kusters, Sanne M M Vermorgen

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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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.

Original language	English
Article number	217
Number of pages	7
Journal	BMC Anesthesiology
Volume	23
DOIs	https://doi.org/10.1186/s12871-023-02147-4
Publication status	Published - 21-Jun-2023

Keywords

Humans
Heparin/therapeutic use
Anticoagulants/therapeutic use
Immunoglobulin Light-chain Amyloidosis/drug therapy
Cardiac Surgical Procedures
Peptide Fragments
Cardiopulmonary Bypass

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Heparin-resistance in AL amyloidosisFinal publisher's version, 2.01 MBLicence: CC BY

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@article{f9378db45b3141f6a6b186deb9882176,

title = "Heparin-resistance in AL amyloidosis: a case report",

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 = "Humans, Heparin/therapeutic use, Anticoagulants/therapeutic use, Immunoglobulin Light-chain Amyloidosis/drug therapy, Cardiac Surgical Procedures, Peptide Fragments, Cardiopulmonary Bypass",

author = "{van Ede}, {Elisabeth S} and Hoeks, {Marlijn P A} and Jan Hofland and Linssen, {Vera D} and {van Kuppevelt}, {Toin H} and Versteeg, {Elly M} and Hafmans, {Theo G} and Arjan Diepstra and Benno Kusters and Vermorgen, {Sanne M M}",

note = "{\textcopyright} 2023. The Author(s).",

year = "2023",

month = jun,

day = "21",

doi = "10.1186/s12871-023-02147-4",

language = "English",

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journal = "BMC Anesthesiology",

issn = "1471-2253",

publisher = "BMC",

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TY - JOUR

T1 - Heparin-resistance in AL amyloidosis

T2 - a case report

AU - van Ede, Elisabeth S

AU - Hoeks, Marlijn P A

AU - Hofland, Jan

AU - Linssen, Vera D

AU - van Kuppevelt, Toin H

AU - Versteeg, Elly M

AU - Hafmans, Theo G

AU - Diepstra, Arjan

AU - Kusters, Benno

AU - Vermorgen, Sanne M M

PY - 2023/6/21

Y1 - 2023/6/21

N2 - 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.

AB - 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.

KW - Humans

KW - Heparin/therapeutic use

KW - Anticoagulants/therapeutic use

KW - Immunoglobulin Light-chain Amyloidosis/drug therapy

KW - Cardiac Surgical Procedures

KW - Peptide Fragments

KW - Cardiopulmonary Bypass

U2 - 10.1186/s12871-023-02147-4

DO - 10.1186/s12871-023-02147-4

M3 - Article

C2 - 37344791

SN - 1471-2253

VL - 23

JO - BMC Anesthesiology

JF - BMC Anesthesiology

M1 - 217

ER -

Heparin-resistance in AL amyloidosis: a case report

Abstract

Keywords

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