Journal
BRITISH JOURNAL OF HAEMATOLOGY
Volume 143, Issue 3, Pages 369-373Publisher
WILEY-BLACKWELL
DOI: 10.1111/j.1365-2141.2008.07327.x
Keywords
immunoglobulin light chains; amyloidosis; melphalan
Categories
Funding
- NIH [CA05826]
- Amyloidosis Research
- Amyloidosis Foundation
- Werner and Elaine Dannheiser Fund for Research on the Biology of Aging of the Lymphoma Foundation
- Demarest Lloyd
- Jr. Foundation
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The treatment of systemic light-chain (AL) amyloidosis with symptomatic cardiac involvement at diagnosis remains a challenge. We report the results of 40 consecutive newly diagnosed AL cardiac patients who were not candidates for stem cell transplant and therefore received monthly oral melphalan and dexamethasone. Median survival was 10.5 months and baseline predictors of survival included gender, troponin I and interventricular septal thickness. The most significant predictor of survival was response to therapy. The haematological response rate was 58% (23/40) with 13% (5/40) complete responses; most responses were noted in < 3 cycles. Achievement of a rapid response to therapy extends survival.
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