Thelansis

 AL amyloidosis, also known as Immunoglobulinic amyloidosis or primary amyloidosis, results from the extracellular deposition of fibrils made up of monoclonal immunoglobulin (Ig) light chain (LC), secreted by a small plasma cell clone that exerts organ toxicity causing organ dysfunction and eventually death. The alteration in the secondary or tertiary structure of an abnormal monoclonal LC results in unstable conformation is the event in the development of Al amyloidosis. The conformational change causes abnormal folding of the LC, which is rich in β leaves and assembles into monomers that stack together to form amyloid fibrils. The typical clinical symptoms are asthenia and dyspnoea, which account for delayed diagnosis. Renal manifestations affect two third of patients, and heart involvement presents in more than 50% of patients as the most severe complication. The combination of Daratumumab, Cyclophosphamide, Bortezomib, and Dexamethasone (Dara-VCd) is currently the novel and pre...