Adriena Sakalová, Lukáš Plank, Dana Škultétyová, Zdenka Štefániková, Mikuláš Hrubiško, Martin Mistrík, Eva Drahošová
Systemic amyloidosis is an incurable metabolic disease caused by monoclonal transformation of plasma cells and production of an insoluble protein – amyloid. Extracellular deposition of amyloid protein to vital organs causes their structural and functional insufficiency with median of survival 1-2 years. Contemporary knowledge about the structure, immunochemical and molecular-genetic properties of amyloid did not resolve the etiopathogenesis of the disease but ameliorated the therapy and prognosis. Plasma cells have a long mitotic index and therefore all clonal processes are characterized with mild plasma cell proliferation (10 % ± 5), paraprotein (λ, IgGλ and rare other) in serum and urine as the source of amyloidogenic light chains. The aim of the study is information about contemporary knowledge. We evaluate also our experience in the group of 32 patients with AL amyloidosis and describe the case report of nephrotic syndrome from infiltration of kidneys with myeloma cells and amyloidosis in the patient with asymptomatic (smoldering) multiple myeloma.