Keywords
primary amyloidosis
free light chains
proteinogram
proteins
How to Cite
Abstract
Introduction: Amyloidosis is a group of diseases that result from misfolding and localized or systemic aggregation of autologous proteins that are deposited in tissues in the form of amyloid fibrils. Primary or free light chain (AL) amyloidosis is the most common form of systemic amyloidosis, with an annual incidence of 0.9 new cases per 100,000 people. Materials and methods: A clinical case of a patient diagnosed with AL associated with IgM Lambda during his stay at the Hospital Interzonal General de Agudos Evita de Lanús. Results: A 47-year-old male patient with a primary diagnosis of heart failure. During hospitalization, it is observed the deposit of amorphous eosinophilic material, congo red positive in the fragment of fibroconnective tissue referred to pathology, thus confirming the diagnosis of amyloidosis with renal and cardiac involvement. While the results in the analysis of the electrophoretic proteinogram in serum and the quantification of free light chains were inconclusive, the presence of a monoclonal component of the IgM Lambda isotype obtained in the immunofixation study in serum, allowed the differential diagnosis of AL. Conclusion: In this patient, the use of the algorithm: serum electrophoretic proteinogram + serum immunofixation + serum Free Light Chains allowed us to arrive at a correct result.
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