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First third-generation CAR T cell application targeting CD19 for the treatment of systemic IgM AL amyloidosis with underlying marginal zone lymphoma

Light chain amyloidosis (AL) is a rare disease caused by the generalized deposition of misfolded free light chains. Patients with immunoglobulin M gammopathy (IgM) and indolent B-cell lymphoma such as marginal zone lymphoma (MZL) may in some instances develop AL amyloidosis. So far, CAR T cells for...

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Main Authors: Korell, Felix (Author) , Schönland, Stefan (Author) , Schmitt, Anita (Author) , Jansen, Madelaine (Author) , Farid, Kiavasch (Author) , Müller-Tidow, Carsten (Author) , Dreger, Peter (Author) , Schmitt, Michael (Author) , Hegenbart, Ute (Author)
Format: Article (Journal) Editorial
Language:English
Published: 14 October 2023
In: Biomarker Research
Year: 2023, Volume: 11, Pages: 1-5
ISSN:2050-7771
DOI:10.1186/s40364-023-00532-2
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1186/s40364-023-00532-2
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Author Notes:Felix Korell, Stefan Schönland, Anita Schmitt, Madelaine Jansen, Kiavasch Farid, Carsten Müller-Tidow, Peter Dreger, Michael Schmitt and Ute Hegenbart
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Summary:Light chain amyloidosis (AL) is a rare disease caused by the generalized deposition of misfolded free light chains. Patients with immunoglobulin M gammopathy (IgM) and indolent B-cell lymphoma such as marginal zone lymphoma (MZL) may in some instances develop AL amyloidosis. So far, CAR T cells for AL amyloidosis have only been reported utilizing the B cell maturation antigen as target, while CD19 has so far not been used in AL amyloidosis. We report the case of a 71-year-old male, diagnosed with systemic AL kappa amyloidosis and MZL, receiving third-generation CAR T cell therapy targeting CD19. Prior treatment included bendamustine/rituximab and cyclophosphamide/ dexamethasone with subsequent autologous stem cell transplantation. CAR T application was well tolerated despite heart and kidney amyloid manifestations, and only early low-grade procedure-specific toxicities were observed. A continuous decrease in IgM, kappa light chains and kappa-to-lambda light chain difference was observed in the patient from day + 30 on, resulting in a deep hematological response six months after treatment. In summary, we present a novel case of CAR T cell treatment with third generation CD19 directed infusion for AL amyloidosis with an underlying secretory active B cell lymphoma, showing that this is an effective treatment modality and can be applied to patients with subsequent AL amyloidosis.
Item Description:Veröffentlicht: 14. Oktober 2023
Gesehen am 14.12.2023
Physical Description:Online Resource
ISSN:2050-7771
DOI:10.1186/s40364-023-00532-2

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