Localized AL amyloidosis

Localized light chain (AL) amyloidosis (amyloidoma) is a rare disease with unclear pathogenesis and undeveloped approaches to therapy. This article presents 3 clinical observations of localized AL amyloidosis with lesions of the respiratory tract, eyes, and soft tissues of the face. Tumor masses consisted of amyloid masses in which single monotypic plasma cells were "embedded". To verify the cell substrate, the method of chromogenic<i> </i><i>in</i><i> </i><i>situ</i> hybridization with probes to immunoglobulin light chains was used. Amyloid typing was performed using FITC-labeled antibodies to kappa and lambda light chains after enzymatic treatment of paraffin sections with pronase. The absence of monoclonal secretion (in two cases) made it difficult to assess the efficacy of the therapy. Taking into account the prevalence and severity of vital organs damage, as well as the recurrent nature of the disease course, systemic combination therapy with targeted drugs was prescribed. Stabilization of the process in all patients was stated during one year of follow-up. Nevertheless, clonreducing therapy in localized AL amyloidosis is not considered as a standard option, its feasibility and efficacy requires further confirmation.

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Publication Details

Journal
Terapevticheskii arkhiv
Published
2025-04-15
DOI
https://doi.org/10.26442/00403660.2025.03.203136
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
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article

Localized AL amyloidosis

Tatyana P. Danilina, И Г Рехтина, А. М. Ковригина, Л. П. Менделеева et al.
Terapevticheskii arkhiv
Amyloidosis: Diagnosis, Treatment, Outcomes
article

Localized AL amyloidosis

Tatyana P. Danilina, И Г Рехтина, А. М. Ковригина, Л. П. Менделеева, Nelly A. Kashchavtseva
article en

Abstract

Localized light chain (AL) amyloidosis (amyloidoma) is a rare disease with unclear pathogenesis and undeveloped approaches to therapy. This article presents 3 clinical observations of localized AL amyloidosis with lesions of the respiratory tract, eyes, and soft tissues of the face. Tumor masses consisted of amyloid masses in which single monotypic plasma cells were "embedded". To verify the cell substrate, the method of chromogenic<i> </i><i>in</i><i> </i><i>situ</i> hybridization with probes to immunoglobulin light chains was used. Amyloid typing was performed using FITC-labeled antibodies to kappa and lambda light chains after enzymatic treatment of paraffin sections with pronase. The absence of monoclonal secretion (in two cases) made it difficult to assess the efficacy of the therapy. Taking into account the prevalence and severity of vital organs damage, as well as the recurrent nature of the disease course, systemic combination therapy with targeted drugs was prescribed. Stabilization of the process in all patients was stated during one year of follow-up. Nevertheless, clonreducing therapy in localized AL amyloidosis is not considered as a standard option, its feasibility and efficacy requires further confirmation.

Terapevticheskii arkhivVol. 97(3)
Sechenov University (RU)
Good health and well-being
Openalex Percentile: Top 93%
Amyloidosis: Diagnosis, Treatment, Outcomes
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