Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review

Abstract Background Non-bacterial thrombotic endocarditis (NBTE) is a rare condition marked by sterile vegetations on cardiac valves, often linked to rheumatologic diseases, autoimmune disorders, and advanced solid malignancies. An early diagnosis and treatment of the associated clinical condition a...

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Main Authors: Fabiana Duarte, Maria Inês Barradas, Ana Raquel Dias, Carlos Faria, Carina Machado, Carolina Pavão
Format: Article
Language:English
Published: BMC 2024-04-01
Series:Cardio-Oncology
Subjects:
Online Access:https://doi.org/10.1186/s40959-024-00226-0
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author Fabiana Duarte
Maria Inês Barradas
Ana Raquel Dias
Carlos Faria
Carina Machado
Carolina Pavão
author_facet Fabiana Duarte
Maria Inês Barradas
Ana Raquel Dias
Carlos Faria
Carina Machado
Carolina Pavão
author_sort Fabiana Duarte
collection DOAJ
description Abstract Background Non-bacterial thrombotic endocarditis (NBTE) is a rare condition marked by sterile vegetations on cardiac valves, often linked to rheumatologic diseases, autoimmune disorders, and advanced solid malignancies. An early diagnosis and treatment of the associated clinical condition are mandatory, although they do not usually eliminate valvular vegetations, making anticoagulation essential to prevent embolic events. Despite variability, the prognosis of NBTE is usually unfavorable due to recurrent embolic events and the severity of the primary condition, typically advanced cancer. Case presentation We present a case of a 57 years-old male who presented to the emergency department with a 5-day history of painful bilateral digital edema and color change episodes (from pallor to cyanosis). Physical examination revealed erythrocyanosis in the distal extremities, prompting consideration of secondary Raynaud syndrome. Despite medical therapy, progressive digital ischemia led to multiple finger amputations. During etiological investigation, anticoagulation tests and autoimmune analysis yielded negative results. A transesophageal echocardiogram was performed, revealing an irregular hyperechogenic mass on the anterior leaflet of the mitral valve without valve dysfunction, and a thoracic computed tomography scan with contrast showed an enlarged right paratracheal lymph node. Histopathological analysis from a transthoracic needle biopsy of the paratracheal lymph node revealed diffuse large B-cell lymphoma. The patient underwent aggressive R-CHOP chemotherapy, achieving a favorable complete response. Conclusion This is a particular case involving the occurrence of NBTE and Raynaud phenomenon as the initial paraneoplastic manifestations in a previously healthy young man. Reports of NBTE associated with lymphoproliferative conditions are quite rare, with fewer than ten cases described in the literature. To our knowledge, this is the first case of NBTE specifically associated with diffuse large B-cell lymphoma.
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spelling doaj.art-ad1a7c1fe2d743988169afe990dda59b2024-04-21T11:31:47ZengBMCCardio-Oncology2057-38042024-04-011011810.1186/s40959-024-00226-0Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature reviewFabiana Duarte0Maria Inês Barradas1Ana Raquel Dias2Carlos Faria3Carina Machado4Carolina Pavão5Cardiology Department, Hospital of Divino Espírito Santo of Ponta DelgadaCardiology Department, Hospital of Divino Espírito Santo of Ponta DelgadaHematology Department, Hospital of Divino Espírito Santo of Ponta Delgada, São Miguel IslandAnatomical Pathology Department, Hospital of Divino Espírito Santo of Ponta Delgada, São Miguel IslandCardiology Department, Hospital of Divino Espírito Santo of Ponta DelgadaHematology Department, Hospital of Divino Espírito Santo of Ponta Delgada, São Miguel IslandAbstract Background Non-bacterial thrombotic endocarditis (NBTE) is a rare condition marked by sterile vegetations on cardiac valves, often linked to rheumatologic diseases, autoimmune disorders, and advanced solid malignancies. An early diagnosis and treatment of the associated clinical condition are mandatory, although they do not usually eliminate valvular vegetations, making anticoagulation essential to prevent embolic events. Despite variability, the prognosis of NBTE is usually unfavorable due to recurrent embolic events and the severity of the primary condition, typically advanced cancer. Case presentation We present a case of a 57 years-old male who presented to the emergency department with a 5-day history of painful bilateral digital edema and color change episodes (from pallor to cyanosis). Physical examination revealed erythrocyanosis in the distal extremities, prompting consideration of secondary Raynaud syndrome. Despite medical therapy, progressive digital ischemia led to multiple finger amputations. During etiological investigation, anticoagulation tests and autoimmune analysis yielded negative results. A transesophageal echocardiogram was performed, revealing an irregular hyperechogenic mass on the anterior leaflet of the mitral valve without valve dysfunction, and a thoracic computed tomography scan with contrast showed an enlarged right paratracheal lymph node. Histopathological analysis from a transthoracic needle biopsy of the paratracheal lymph node revealed diffuse large B-cell lymphoma. The patient underwent aggressive R-CHOP chemotherapy, achieving a favorable complete response. Conclusion This is a particular case involving the occurrence of NBTE and Raynaud phenomenon as the initial paraneoplastic manifestations in a previously healthy young man. Reports of NBTE associated with lymphoproliferative conditions are quite rare, with fewer than ten cases described in the literature. To our knowledge, this is the first case of NBTE specifically associated with diffuse large B-cell lymphoma.https://doi.org/10.1186/s40959-024-00226-0Non-bacterial thrombotic endocarditisNon-Hodgkin lymphomaRaynaud phenomenonVascular surgeryCase report
spellingShingle Fabiana Duarte
Maria Inês Barradas
Ana Raquel Dias
Carlos Faria
Carina Machado
Carolina Pavão
Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review
Cardio-Oncology
Non-bacterial thrombotic endocarditis
Non-Hodgkin lymphoma
Raynaud phenomenon
Vascular surgery
Case report
title Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review
title_full Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review
title_fullStr Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review
title_full_unstemmed Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review
title_short Nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy: case report and literature review
title_sort nonbacterial thrombotic endocarditis of mitral valve associated with a lymphoproliferative malignancy case report and literature review
topic Non-bacterial thrombotic endocarditis
Non-Hodgkin lymphoma
Raynaud phenomenon
Vascular surgery
Case report
url https://doi.org/10.1186/s40959-024-00226-0
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