Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature

IntroductionPrimary immune thrombocytopenia is an autoimmune bleeding disorder characterized by variable immune-mediated platelet destruction. These patients have reported thrombotic complications, both venous and arterial, in addition to bleeding. Splenectomy and thrombopoietin receptor agonists ar...

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Main Authors: Roberto Castelli, Enrico Atzori, Alessandro Palmerio Delitala, Salvatore Antonio Masala, Valentina Micheluzzi, Enrico Ponti, Giuseppe Sanna, Dante Castro, Antonio Gidaro, Mattia Donadoni, Roberto Manetti, Pierluigi Merella, Nicia Isabella Profili, Andrea Maria D'Armini, Gavino Casu
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Language:English
Published: Frontiers Media S.A. 2025-02-01
Series:Frontiers in Cardiovascular Medicine
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Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2025.1508574/full
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author Roberto Castelli
Enrico Atzori
Alessandro Palmerio Delitala
Salvatore Antonio Masala
Valentina Micheluzzi
Enrico Ponti
Enrico Ponti
Giuseppe Sanna
Dante Castro
Antonio Gidaro
Mattia Donadoni
Roberto Manetti
Pierluigi Merella
Nicia Isabella Profili
Andrea Maria D'Armini
Gavino Casu
Gavino Casu
author_facet Roberto Castelli
Enrico Atzori
Alessandro Palmerio Delitala
Salvatore Antonio Masala
Valentina Micheluzzi
Enrico Ponti
Enrico Ponti
Giuseppe Sanna
Dante Castro
Antonio Gidaro
Mattia Donadoni
Roberto Manetti
Pierluigi Merella
Nicia Isabella Profili
Andrea Maria D'Armini
Gavino Casu
Gavino Casu
author_sort Roberto Castelli
collection DOAJ
description IntroductionPrimary immune thrombocytopenia is an autoimmune bleeding disorder characterized by variable immune-mediated platelet destruction. These patients have reported thrombotic complications, both venous and arterial, in addition to bleeding. Splenectomy and thrombopoietin receptor agonists are recommended for patients who do not respond to steroids or immunosuppressive treatments. Chronic thromboembolic pulmonary hypertension is a rare disease that results from a persistent, organized thromboembolic obstruction of the pulmonary arteries due to an incompletely resolved pulmonary embolism.Case presentationsWe report two cases of chronic thromboembolic pulmonary hypertension induced by a thrombotic mechanism after treatment with splenectomy and Eltrombopag, a thrombopoietin receptor agonist, for refractory primary immune thrombocytopenia. Consequently, the patients were referred for surgical pulmonary thromboendarterectomy therapy as suggested.ConclusionIn older patients, those with a history of thrombotic manifestations, or those with high-risk factors, clinicians should evaluate and monitor the risk of thrombotic events and chronic thromboembolic pulmonary hypertension when treating primary immune thrombocytopenia with splenectomy and Eltrombopag.
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spelling doaj-art-7005e65c196d47a5ba54a99d594506292025-02-12T07:26:29ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2025-02-011210.3389/fcvm.2025.15085741508574Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literatureRoberto Castelli0Enrico Atzori1Alessandro Palmerio Delitala2Salvatore Antonio Masala3Valentina Micheluzzi4Enrico Ponti5Enrico Ponti6Giuseppe Sanna7Dante Castro8Antonio Gidaro9Mattia Donadoni10Roberto Manetti11Pierluigi Merella12Nicia Isabella Profili13Andrea Maria D'Armini14Gavino Casu15Gavino Casu16Department of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyClinical and Interventional Cardiology, University Hospital, Sassari, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyClinical and Interventional Cardiology, University Hospital, Sassari, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyClinical and Interventional Cardiology, University Hospital, Sassari, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyDepartment of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, ItalyDepartment of Biomedical and Clinical Sciences Luigi Sacco, Luigi Sacco Hospital, University of Milan, Milan, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyClinical and Interventional Cardiology, University Hospital, Sassari, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyCardiac Surgery, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDepartment of Medicine, Surgery and Pharmacy, University of Sassari, Sassari, ItalyClinical and Interventional Cardiology, University Hospital, Sassari, ItalyIntroductionPrimary immune thrombocytopenia is an autoimmune bleeding disorder characterized by variable immune-mediated platelet destruction. These patients have reported thrombotic complications, both venous and arterial, in addition to bleeding. Splenectomy and thrombopoietin receptor agonists are recommended for patients who do not respond to steroids or immunosuppressive treatments. Chronic thromboembolic pulmonary hypertension is a rare disease that results from a persistent, organized thromboembolic obstruction of the pulmonary arteries due to an incompletely resolved pulmonary embolism.Case presentationsWe report two cases of chronic thromboembolic pulmonary hypertension induced by a thrombotic mechanism after treatment with splenectomy and Eltrombopag, a thrombopoietin receptor agonist, for refractory primary immune thrombocytopenia. Consequently, the patients were referred for surgical pulmonary thromboendarterectomy therapy as suggested.ConclusionIn older patients, those with a history of thrombotic manifestations, or those with high-risk factors, clinicians should evaluate and monitor the risk of thrombotic events and chronic thromboembolic pulmonary hypertension when treating primary immune thrombocytopenia with splenectomy and Eltrombopag.https://www.frontiersin.org/articles/10.3389/fcvm.2025.1508574/fullprimary immune thrombocytopeniathrombopoietin receptors agonistseltrombopagsplenectomychronic thromboembolic pulmonary hypertensionpulmonary thromboendarterectomy
spellingShingle Roberto Castelli
Enrico Atzori
Alessandro Palmerio Delitala
Salvatore Antonio Masala
Valentina Micheluzzi
Enrico Ponti
Enrico Ponti
Giuseppe Sanna
Dante Castro
Antonio Gidaro
Mattia Donadoni
Roberto Manetti
Pierluigi Merella
Nicia Isabella Profili
Andrea Maria D'Armini
Gavino Casu
Gavino Casu
Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature
Frontiers in Cardiovascular Medicine
primary immune thrombocytopenia
thrombopoietin receptors agonists
eltrombopag
splenectomy
chronic thromboembolic pulmonary hypertension
pulmonary thromboendarterectomy
title Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature
title_full Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature
title_fullStr Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature
title_full_unstemmed Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature
title_short Case Report: Dangerous liaisons between splenectomy and eltrombopag-induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia: report of two cases and review of the literature
title_sort case report dangerous liaisons between splenectomy and eltrombopag induced chronic thromboembolic pulmonary hypertension in patients with immune thrombocytopenia report of two cases and review of the literature
topic primary immune thrombocytopenia
thrombopoietin receptors agonists
eltrombopag
splenectomy
chronic thromboembolic pulmonary hypertension
pulmonary thromboendarterectomy
url https://www.frontiersin.org/articles/10.3389/fcvm.2025.1508574/full
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