Factors impeding organ recovery despite a deep haematological response in patients with systemic <scp>AL</scp> amyloidosis

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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Publication Details

Journal
British Journal of Haematology
Published
2024-09-14
DOI
https://doi.org/10.1111/bjh.19766
Citations
5
Primary Topic
Amyloidosis: Diagnosis, Treatment, Outcomes
Type
article
Field-Weighted Citation Impact
1.17
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article

Factors impeding organ recovery despite a deep haematological response in patients with systemic AL amyloidosis

Natasha Burke, Vaishali Sanchorawala, Lisa Mendelson, Tracy Joshi et al.
5 citations
British Journal of Haematology
Amyloidosis: Diagnosis, Treatment, Outcomes
1.17
article

Factors impeding organ recovery despite a deep haematological response in patients with systemic AL amyloidosis

Natasha Burke, Vaishali Sanchorawala, Lisa Mendelson, Tracy Joshi, Andrew Staron
article en
5 citations

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.

British Journal of HaematologyVol. 205(6)
Boston University (US), Boston Medical Center (US), Amyloidosis Foundation (US)
Good health and well-being
Openalex Percentile: Top 22%
Amyloidosis: Diagnosis, Treatment, Outcomes
1.17
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