期刊
BRITISH JOURNAL OF HAEMATOLOGY
卷 143, 期 3, 页码 369-373出版社
WILEY-BLACKWELL
DOI: 10.1111/j.1365-2141.2008.07327.x
关键词
immunoglobulin light chains; amyloidosis; melphalan
类别
资金
- 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
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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