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Systemic AL amyloidosis due to non-Hodgkin's lymphoma: an unusual clinicopathologic association by A. D. Cohen; P. Zhou; Q. Xiao; M. Fleisher; N. Kalakonda; T. Akhurst; D. A. Chitale; C. Moscowitz; M. V. Dhodapkar; J. Teruya-Feldstein; D. Filippa; R. L. Comenzo is a Medicine article available to read on EtoBox.
What is Systemic AL amyloidosis due to non-Hodgkin's lymphoma: an unusual clinicopathologic association about?
## 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 __V__~L~) 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 employ
Who reads Systemic AL amyloidosis due to non-Hodgkin's lymphoma: an unusual clinicopathologic association?
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- Author
- A. D. Cohen; P. Zhou; Q. Xiao; M. Fleisher; N. Kalakonda; T. Akhurst; D. A. Chitale; C. Moscowitz; M. V. Dhodapkar; J. Teruya-Feldstein; D. Filippa; R. L. Comenzo
- Publisher
- John Wiley and Sons; Wiley (Blackwell Publishing); Blackwell Publishing Inc.; Wiley (ISSN 0007-1048)
- Published
- 2004
- Language
- EN
- Field
- Medicine (Health Sciences)