Two distinct syndromes of lymphoma-associated AL amyloidosis: a case series and review of the literature

Abstract

Light chain (AL) amyloidosis has a rare association with non-Hodgkin lymphoma (NHL). Both peritumoral and systemic AL amyloidosis have been reported, but a detailed description of these syndromes is lacking. We describe 10 patients with lymphoma associated AL amyloidosis. NHL patients with peritumoral amyloidosis had low or undetectable levels of monoclonal (M) protein, mostly single organ involvement(lung or soft tissue), and underlying extranodal marginal zone lymphoma, mucosa associated lymphoid tissue subtype. NHL patients with systemic amyloidosis had high levels of M-protein, multiorgan involvement with frequent cardiac involvement, and predominantly underlying lymphoplasmacytic lymphoma. Systemic amyloidosis was associated with inferior outcomes