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. 2021 Dec;21(12):e946-e959.
doi: 10.1016/j.clml.2021.07.003. Epub 2021 Jul 19.

Light Chain Amyloidosis (AL) Associated With B Cell Lymphoma a Single Center Experience

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Light Chain Amyloidosis (AL) Associated With B Cell Lymphoma a Single Center Experience

Alfredo De la Torre et al. Clin Lymphoma Myeloma Leuk. 2021 Dec.

Abstract

Introduction: Light chain (AL) amyloidosis and B-cell lymphoma represent 5% to 7% of all AL, Systemic amyloidosis, deposits in sites remote from the underlying lymphoma, and peritumoral amyloidosis deposition is within the immediate vicinity.

Materials and methods: We conducted a retrospectively study to identify and describe AL with B cell lymphoma at Princess Margaret Cancer Center from 01 January 1997 to 31 July 2019.

Results: Thirty-five patients with AL and lymphoma, an incidence of 6, 2%, median age of diagnosis of 66 (range 47 to 86), majority male, most had underlying Waldestrom's Macroglobulinemia. 21 patients with peritumoral AL (PAL), and 15 with systemic AL. 42.8% of the patients had major organ involvement. 35% got treatment with Rituximab with alkylator, 20% received proteasome inhibitors, 17% patients were on a watch and wait approach, amyloid response showed very good partial response > 45.8%, and lymphoma ORR was 42.8%, with a median follow up of 31.5 months. A 36 month overall survival (OS) and progression-free survival (PFS) showed worse outcomes for heart involvement OS (P = .002), PFS (0.057) and IgM subtype OS (P = .02), PFS (0.01).

Conclusion: We have shown adverse outcome with IgM AL and to document a differences in OS and PFS not previously reported for PAL.

Keywords: Peritumoral amyloidosis; Peritumoral disease; Plasma cell disorder; Systemic amyloidosis; Systemic disease.

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