Coexistence of Wild-Type Transthyretin Cardiac Amyloidosis and Monoclonal Gammopathy in a Patient With Polycythemia Vera

Polycythemia vera is a type of myeloproliferative neoplasm characterized by panmyelosis, with a predominant increase in erythrocyte mass. In contrast, monoclonal gammopathy of undetermined significance is classified as a premalignant plasma cell disorder and is characterized by elevated levels of monoclonal γ-globulin without associated bone lesions or clinical symptoms. Here, we present a case of the concurrent presence of wild-type transthyretin cardiac amyloidosis—not the amyloid light-chain type—and monoclonal gammopathy of undetermined significance in a patient with polycythemia vera. This case underscores the crucial importance of preforming histologic subtyping to ensure accurate diagnosis in older patients with undiagnosed cardiac amyloidosis.

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Publication Details

Journal
Annals of Internal Medicine Clinical Cases
Published
2026-02-01
DOI
https://doi.org/10.7326/aimcc.2025.0923
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
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article

Coexistence of Wild-Type Transthyretin Cardiac Amyloidosis and Monoclonal Gammopathy in a Patient With Polycythemia Vera

Masaki Maruta, Haruhiko Higashi, Amane Otoi, AKINORI HIGAKI et al.
Annals of Internal Medicine Clinical Cases
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Coexistence of Wild-Type Transthyretin Cardiac Amyloidosis and Monoclonal Gammopathy in a Patient With Polycythemia Vera

Masaki Maruta, Haruhiko Higashi, Amane Otoi, AKINORI HIGAKI, Osamu Yamaguchi
article en

Abstract

Polycythemia vera is a type of myeloproliferative neoplasm characterized by panmyelosis, with a predominant increase in erythrocyte mass. In contrast, monoclonal gammopathy of undetermined significance is classified as a premalignant plasma cell disorder and is characterized by elevated levels of monoclonal γ-globulin without associated bone lesions or clinical symptoms. Here, we present a case of the concurrent presence of wild-type transthyretin cardiac amyloidosis—not the amyloid light-chain type—and monoclonal gammopathy of undetermined significance in a patient with polycythemia vera. This case underscores the crucial importance of preforming histologic subtyping to ensure accurate diagnosis in older patients with undiagnosed cardiac amyloidosis.

Annals of Internal Medicine Clinical CasesVol. 5(2)
Ehime University (JP)
Openalex Percentile: Top 69%
Amyloidosis: Diagnosis, Treatment, Outcomes
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