Peritoneal ultrafiltration therapy as an alternative for prognostic improvement in a case of AL amyloidosis light chain with cardiac involvement

Abstract

Amyloidosis is a systemic disease characterized by poor protein processing and systemic deposition. Amyloid material shows positive staining for "Congo red" under light microscopy. Many proteins can lead to amyloid deposition. The most frequent forms of this disease are primary amyloidosis (AL) due to alpha light chains and amyloidosis due to transthyretin. In alpha light chain AL amyloidosis, there is an exaggerated clonal proliferation of a single alpha light chain subtype. The kidney is the most frequently affected organ, followed by the heart. Kidney protein deposition can be at the glomerular or tubular level. The most frequent form is at the glomerular level, causing proteinuria, nephrotic syndrome in some cases, and kidney dysfunction. In the heart, the deposition of these proteins causes left ventricular hypertrophy, valvular involvement, and cardiac rhythm disorders, all of which contribute to an evolution towards heart failure with preserved left ventricle ejection fraction (LVEF). Cardiac involvement, specifically when it results in congestive symptoms, determines this disease's prognosis, morbidity, and mortality. The congestive symptoms will mark the course of the disease, as occurs in most patients with heart failure, regardless of the etiology that causes it. Refractory congestion occurs when congestive signs and symptoms do not respond to the usual treatment with diuretics. Peritoneal ultrafiltration has been positioned as an alternative for the clinical management of refractory systemic congestion. Peritoneal ultrafiltration, unlike hemodialysis, allows a more continuous and physiological depletion with less stress on the myocardium and better preservation of residual renal function.  We present the case of a patient studied for heart failure with preserved LVEF and chronic kidney disease with a final diagnosis of AL light chain amyloidosis. Despite receiving chemotherapy treatment, he presented a rapid progression of the disease with severe cardiac involvement, the development of clinical congestion refractory to medical treatment, and minimal quality of life. He was proposed to participate in the peritoneal ultrafiltration program as a therapeutic measure for congestive symptoms. After being included in the treatment, this technique allowed him to control his symptoms and improve the prognosis of his disease in the short term, significantly improving his quality of life.