1,721,389 research outputs found

    AL amyloidosis: from molecular mechanisms to targeted therapies

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    https://www.ncbi.nlm.nih.gov/pubmed/2922223

    Introduction

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    https://www.ncbi.nlm.nih.gov/pubmed/3309943

    Redirecting proteoxicity

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    https://www.ncbi.nlm.nih.gov/pubmed/3283949

    Two-hit strategy for treating AL amyloidosis?

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    In this issue of Blood, Edwards et al report the results of a phase 1a/1b trial of passive antiamyloid immunotherapy with the monoclonal antibody CAEL-101 in patients with light chain (LC) amyloidosis with persistent organ dysfunction despite response to previous chemotherapy. Two-thirds of evaluable patients attained cardiac response and 20% reached renal response at a median time of only 3 weeks. Treatment was well tolerated. These findings open new therapeutic opportunities and create new challenges.(1

    Light Chains With Heavy Effects

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    https://www.ncbi.nlm.nih.gov/pubmed/3162957

    Monoclonal IgM-related AL amyloidosis

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    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

    Improved outcomes for kidney transplantation in AL amyloidosis: impact on practice

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    Effective therapies for Ig light chain (AL) amyloidosis has led to an increasing proportion of patients with end-stage renal disease requiring renal replacement therapy, yet kidney transplantation is seldom performed in this setting due to concerns of renal and extrarenal disease progression. Angel-Korman et al. report unprecedented positive long-term outcomes in the largest series of kidney transplantation in AL amyloidosis providing the basis for a more proactive approach to this procedure
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