Systemic AL amyloidosis due to non-Hodgkin's lymphoma: An unusual clinicopathologic association Journal Article
| Authors: | Cohen, A. D.; Zhou, P.; Xiao, Q.; Fleisher, M.; Kalakonda, N.; Akhurst, T.; Chitale, D. A.; Moskowitz, C.; Dhodapkar, M. V.; Teruya-Feldstein, J.; Filippa, D.; Comenzo, R. L. |
| Article Title: | Systemic AL amyloidosis due to non-Hodgkin's lymphoma: An unusual clinicopathologic association |
| Abstract: | 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 V L 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: | adult; clinical article; controlled study; treatment outcome; aged; middle aged; clinical feature; histopathology; antineoplastic agents; rituximab; positron emission tomography; antineoplastic agent; disease association; amyloidosis; stem cell transplantation; plasma cell; cd20 antigen; antibodies, monoclonal; correlation analysis; nonhodgkin lymphoma; immunoglobulin variable region; non-hodgkin's lymphoma; lymphoma, non-hodgkin; immunoglobulin light chain; immunoglobulin light chains; tomography, emission-computed; syndecan 1; lymphoid cell; genes, immunoglobulin; humans; human; male; female; priority journal; article |
| Journal Title: | British Journal of Haematology |
| Volume: | 124 |
| Issue: | 3 |
| ISSN: | 0007-1048 |
| Publisher: | John Wiley & Sons |
| Date Published: | 2004-02-01 |
| Start Page: | 309 |
| End Page: | 314 |
| Language: | English |
| DOI: | 10.1046/j.1365-2141.2003.04779.x |
| PROVIDER: | scopus |
| PUBMED: | 14717777 |
| DOI/URL: | |
| Notes: | Br. J. Haematol. -- Cited By (since 1996):34 -- Export Date: 16 June 2014 -- CODEN: BJHEA -- Source: Scopus |
