AL amyloidosis treatment relies on cytoreductive, PC-directed chemotherapy and/or immune-therapy with the goal of achieving rapid and deep hematologic remission to halt progression of end-organ damage. A high index of clinical suspicion is crucial to rapidly pursue diagnostic studies and definitive tissue diagnosis in an effort to preserve organ function and maximize likelihood of short-term survival. FLC amyloid deposition can occur in any organ except the central nervous system, underscoring the variable and often complex clinical presentation. In immunoglobulin light chain (AL) amyloidosis, the amyloidogenic protein is a misfolded immunoglobulin (Ig) free light chain (FLC), typically produced by clonal plasma cells (PC), less often by a more immature B cell neoplasm ( 2). The identity of the precursor protein distinguishes the various amyloidoses and guides organ tropism and the therapeutic approach ( 1).
Amyloidoses are a heterogeneous group of diseases characterized by organized deposition of a misfolded protein in repetitive β-pleated sheets in target organs.
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