Amyloidosis related to monoclonal B-lymphocyte dyscrasia: A systematic and epidemiological view

Abstract

Amyloidosis caused by monoclonal dyscrasia of B lymphocytes is a complex and severe disease, characterized by the progressive accumulation of amyloid material resulting from the incorrect folding of immunoglobulin light chains κ or λ, which are deposited in tissues. It is a pathology that can manifest itself systemically or locally, depending on the level and location of the accumulation, being more serious and prevalent in vital organs, such as the heart and kidneys. Clinically, patients present with a variety of generic signs, including edema, renal and cardiac dysfunction, and as the level of accumulation progresses, the characteristic signs of the disease, such as macroglossia, periorbital purpura, and changes in skin texture, become evident and aid in the presumptive diagnosis. However, the effective diagnosis of AL amyloidosis is complex, based on clinical observation and confirmed by auxiliary diagnostic methods, the most effective being local biopsy, together with Congo red staining, which is particularly sensitive to this type of accumulation, with the ability to birefringence under polarized light, showing amyloid accumulations of greenish-brown amyloid. The research used the bibliographic methodology, searching the literature of the last five years in databases such as PUBMED and Google Scholar. The data collected show the heterogeneity of the disease and its high prevalence and incidence in countries with developed health systems, which contrasts with the difficulty of diagnosis and treatment in less developed regions. The study highlights the urgent need to develop more effective treatments and new therapeutic approaches to combat the progression of the disease, which continues to have a high mortality rate and a significant impact on patients' quality of life.