Amylose AL

Light chain amyloidosis (AL) is the most common subtype of amyloidosis except wild type tranthyretine amyloidosis and is caused by the deposition of misfolded monoclonal light chains of immunoglobulins produced by a monoclonal B cell, mainly of plasma cell origin. Affected patients may present with amyloidosis alone or in association with other plasma cell or lymphoid dyscrasias (multiple myeloma, Waldenström macroglobulinemia or other B lymphoma). Diagnosis of amyloidosis is histological. Cardiac and renal involvement are the most frequent and present in nearly two thirds of patients as hypertrophic heart disease and/or nephrotic syndrome, respectively. AL amyloidosis is a clonal plasma cell disorder and is treated by chemotherapy dedicated to eradicate the underlying clone. Assessment of the severity of the disease with the Mayo Clinic score is used to guide the choice of treatment. First-line treatment combine bortezomib, cyclophosphamide or melphalan and dexamethasone for severe cases, plus or minus daratumumab, an anti-CD-38 monoclonal antibody, following the excellent results of the ANDROMEDA phase 3 study; mild cases can still benefit from melphalan and dexamethasone.