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Factors impeding organ recovery despite a deep haematological response in patients with systemic AL amyloidosis
Andrew Staron1,2, Lisa M Mendelson1, Tracy Joshi1
1Amyloidosis Center, Boston University Chobanian & Avedisian School of Medicine, Boston, Massachusetts, USA.
Abstract:
Patients with AL amyloidosis can have persistent organ dysfunction despite achieving a haematological complete response (hemCR). We aimed to identify factors for organ non-response among 143 patients who achieved hemCR for ≥6 months. Kidney, heart and liver non-response were observed in 40/117 (34%), 19/68 (28%) and 3/17 (18%) patients respectively. Predisposing factors varied by organ system. Kidney non-responders had more advanced organ dysfunction at diagnosis, whereas heart non-responders had disproportionately more lambda-typic amyloidogenic light chains. Most patients without an apparent reason for organ non-response had detectable residual clonal disease. The interplay of factors impeding organ recovery in AL amyloidosis is complex.
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