Predictors of survival in patients with systemic light-chain amyloidosis and cardiac involvement initially ineligible for stem cell transplantation and treated with oral melphalan and dexamethasone Journal Article


Authors: Lebovic, D.; Hoffman, J.; Levine, B. M.; Hassoun, H.; Landau, H.; Goldsmith, Y.; Maurer, M. S.; Steingart, R. M.; Cohen, A. D.; Comenzo, R. L.
Article Title: Predictors of survival in patients with systemic light-chain amyloidosis and cardiac involvement initially ineligible for stem cell transplantation and treated with oral melphalan and dexamethasone
Abstract: 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. © 2008 The Authors.
Keywords: survival; adult; clinical article; treatment outcome; treatment response; aged; aged, 80 and over; middle aged; survival analysis; acute granulocytic leukemia; thalidomide; drug dose reduction; monotherapy; patient selection; multiple cycle treatment; drug administration schedule; dexamethasone; melphalan; amyloidosis; stem cell transplantation; drug fatality; survival time; drug therapy, combination; add on therapy; kidney function; proton pump inhibitor; ciprofloxacin; predictor variable; sex difference; cardiomyopathies; sex factors; administration, oral; immunoglobulin light chain; immunoglobulin light chains; granulocyte colony stimulating factor; troponin i; heart amyloidosis; fluconazole; glucocorticoids; heart ventricle septum; hematological parameters; systemic light chain amyloidosis
Journal Title: British Journal of Haematology
Volume: 143
Issue: 3
ISSN: 0007-1048
Publisher: John Wiley & Sons  
Date Published: 2008-11-01
Start Page: 369
End Page: 373
Language: English
DOI: 10.1111/j.1365-2141.2008.07327.x
PUBMED: 18691169
PROVIDER: scopus
DOI/URL:
Notes: --- - "Cited By (since 1996): 19" - "Export Date: 17 November 2011" - "CODEN: BJHEA" - "Source: Scopus"
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MSK Authors
  1. Adam D Cohen
    44 Cohen
  2. Raymond L Comenzo
    115 Comenzo
  3. James Edward Hoffman
    10 Hoffman
  4. Richard M Steingart
    174 Steingart
  5. Hani Hassoun
    329 Hassoun
  6. Heather Jolie Landau
    419 Landau
  7. Beth Mara Levine
    2 Levine