Abstract
Systemic amyloid light chain (AL) amyloidosis is a rare protein deposition disease caused by a clonal B cell disorder of the bone marrow. The underlying diseases can be plasma cell disorders (monoclonal gammopathy of clinical significance, smoldering or symptomatic myeloma) or B cell non-Hodgkin’s lymphoma (e.g., Waldenstrom’s disease or marginal zone lymphoma) with secretory activity. It is crucial to characterize the underlying disease very precisely as the treatment of AL amyloidosis is directed against the (often small) B cell clone. Finally, the detection of cytogenetic aberrations of the plasma cell clone will likely play an important role for choosing an effective drug in the near future.
Highlights
Systemic amyloid light-chain (AL-) amyloidosis is a rare monoclonal B cell disorder with poor prognosis due to the production of free light chains leading to amyloid deposition in most organs [1]
In about 90% of amyloid light chain (AL) patients the underlying disease is derived from clonal plasma cells (AL-PC), therapeutic strategies are mostly adapted from established myeloma regimens
Daratumumab in combination with CyBorD was approved in the US and Europe for newly diagnosed patients with AL amyloidosis
Summary
Systemic amyloid light-chain (AL-) amyloidosis is a rare monoclonal B cell disorder with poor prognosis due to the production of free light chains leading to amyloid deposition in most organs [1]. Most patients succumb to advanced heart failure. The only evidencebased and recently approved therapy is cytoreduction of the underlying monoclonal aberrant plasma or B cell population to reduce or even stop the production of amyloid precursors leading to an improvement of organ functions. In about 90% of AL patients the underlying disease is derived from clonal plasma cells (AL-PC), therapeutic strategies are mostly adapted from established myeloma regimens. In about 10% the underlying clone is a B cell clone [1] and in such cases drugs established in those lymphomas are used.
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