Monoclonal immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 5%–7% of all AL amyloidosis cases, is a distinct clinical entity that poses specific challenges to clinicians. Several studies reported that although there is a substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. A recent collaborative study from three European referral centers, defined that cardiac involvement, advanced Mayo disease stage, neuropathic, and liver involvement were independent factors that had impact on survival in IgM-AL amyloidosis patients. Once the diagnosis of amyloidosis is made, correct amyloid typing is necessary to design appropriate therapy and follow-up. Treatment is focused on the suppression of the clone, and fast reduction of the circulating free light chains. New drugs targeting the amyloid deposits will be used in combination with anti-clone therapies.
Monoclonal IgM-related AL amyloidosis
Milani P.;Merlini G.
2016-01-01
Abstract
Monoclonal immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 5%–7% of all AL amyloidosis cases, is a distinct clinical entity that poses specific challenges to clinicians. Several studies reported that although there is a substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. A recent collaborative study from three European referral centers, defined that cardiac involvement, advanced Mayo disease stage, neuropathic, and liver involvement were independent factors that had impact on survival in IgM-AL amyloidosis patients. Once the diagnosis of amyloidosis is made, correct amyloid typing is necessary to design appropriate therapy and follow-up. Treatment is focused on the suppression of the clone, and fast reduction of the circulating free light chains. New drugs targeting the amyloid deposits will be used in combination with anti-clone therapies.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
