What is the prognosis for primary amyloidosis (AL) and what is the evidence for management?

PROGNOSIS:
Kyle RA, et al. Long-term survival (10 years or more) in 30 patients with primary amyloidosis.
Blood. 1999 Feb 1;93(3):1062-6. PMID: 9920856
Highlights:
Fig. 1 Survival curve for 810 patients with systemic amyloidosis
Table 2 Comparison of patients survival

Br J Haematol. 2004 Jun;125(6):681-700. Guidelines on the diagnosis and management of AL amyloidosis.
Highlights:
These guidelines include prognostic factors at the bottom of p. 686.

THERAPY:
Br J Haematol. 2004 Jun;125(6):681-700. Guidelines on the diagnosis and management of AL amyloidosis.
Recommendations and evidence for chemotherapy and stem cell transplantation, pp. 687-693
Summary of treatment recommendations, pp.693 and following.

Recent trials
Jaccard A, et al. High-dose melphalan versus melphalan plus dexamethasone for AL amyloidosis. N Engl J Med. 2007 Sep 13;357(11):1083-93.
Highlights:
RCT 100 pts in two groups
Fig. 2, Survival curves by hematologic responses and treatment group
Fig. 3, Survival according to risk group and according to treatment group among patients with low-risk disease

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About Amy

Clinical Informationist at EUH Branch Library
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