Systemic AL amyloidosis due to non-Hodgkin's lymphoma: an unusual clinicopathologic association

Authors: Cohen A.D.1; Zhou P.2; Xiao Q.3; Fleisher M.4; Kalakonda N.2; Akhurst T.5; Chitale D.A.3; Moscowitz C.1; Dhodapkar M.V.; Teruya-Feldstein J.3; Filippa D.3; Comenzo R.L.

Source: British Journal of Haematology, Volume 124, Number 3, February 2004 , pp. 309-314(6)

Publisher: Blackwell Publishing

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Abstract:

Summary

Systemic AL amyloidosis (AL) is a disorder in which light chains form fibrillar deposits, leading to organ dysfunction and death. Rarely, AL has been associated with non-Hodgkin's lymphoma (NHL), although this association has not been well characterized. We report a series of six patients with AL associated with NHL, primarily lymphoplasmacytic lymphoma. Organ involvement was variable, with frequent bulky lymphadenopathy and visceral cavity deposits, but no cardiac involvement. Positron emission tomography scans were negative. Bone marrow and lymph node biopsies showed a mixed population of CD20+ lymphoid and CD138+ plasma cells. Serum free light chains were elevated, and correlated with response to therapy. Immunoglobulin light chain variable region (Ig VL) germline gene use was typical for AL, reflecting previously observed correlations between germline gene use and organ tropism. Five patients received rituximab-based therapies with two responses. Two patients underwent autologous stem cell transplantation with one complete haematological response. Four patients survive at 10–132 months from diagnosis. AL with NHL has distinctive clinical features but employs the same Ig VL gene repertoire as AL with clonal plasma cell dyscrasias. Serial serum free light chain levels are useful for tracking response to therapy. Treatments aimed at both lymphoid and plasma cell components appear warranted.

Keywords: amyloidosis; non-Hodgkin's lymphoma; immunoglobulin light chains; rituximab

Document Type: Research article

DOI: 10.1046/j.1365-2141.2003.04779.x

Affiliations: 1: Department of Medicine, Memorial Sloan-Kettering Cancer Center 2: Sloan-Kettering Institute 3: Department of Pathology 4: Department of Clinical Laboratories 5: Department of Radiology, Memorial Sloan-Kettering Cancer Center

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