Multisystem involvement associated with AL-amyloidosis: A clinical case

Abstract

AL-amyloidosis is a severe multisystem disease that, in the absence of timely diagnosis and treatment, determines a negative prognosis for patients' quality of life and survival. We present a clinical case of a patient with long-standing chronic kidney disease and severe hypotension. An in-depth assessment of the echocardiographic picture, considering the "red flags" of amyloidosis (left ventricular myocardial hypertrophy ≥ 12 mm combined with diastolic dysfunction), as well as gadolinium-enhanced cardiac magnetic resonance imaging, revealed features characteristic of amyloid cardiomyopathy. Laboratory methods allowed the detection of pathological secretion of lambda light chains and the exclusion of multiple myeloma. Histological examination provided evidence of multisystem amyloid deposits in the gastrointestinal mucosa and renal parenchyma. Consequently, a diagnosis of AL-amyloidosis was established, enabling prompt initiation of daratumumab therapy aimed at suppressing clonal light chain secretion and, accordingly, reducing amyloid protein production. This may be decisive for increasing survival and maintaining a high quality of life for the patient. The value of this clinical case lies in the necessity of a thorough evaluation of patients presenting with a combination of chronic kidney disease and marked myocardial hypertrophy. The use of amyloidosis "red flags" and a multimodal diagnostic approach in clinical practice increases the detectability of AL-amyloidosis and positively impacts the survival and quality of life of patients with this disease.