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

被引：90

作者：

Lebovic, D. ^{[2
]}

Hoffman, J. ^{[1
]}

Levine, B. M. ^{[1
]}

Hassoun, H. ^{[1
]}

Landau, H. ^{[1
]}

Goldsmith, Y. ^{[3
]}

Maurer, M. S. ^{[4
]}

Steingart, R. M. ^{[3
]}

Cohen, A. D. ^{[1
]}

Comenzo, R. L. ^{[1
]}

机构：

[1] Mem Sloan Kettering Canc Ctr, Dept Med, Div Hematol Oncol, New York, NY 10021 USA

[2] New York Hosp, Dept Med, Weill Cornell Sch Med, New York, NY 10021 USA

[3] Mem Sloan Kettering Canc Ctr, Dept Med, Div Cardiol Serv, New York, NY 10021 USA

[4] Columbia Univ, Med Ctr, Div Cardiol, New York, NY USA

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2008年 / 143卷 / 03期

关键词：

immunoglobulin light chains; amyloidosis; melphalan;

D O I：

10.1111/j.1365-2141.2008.07327.x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：369 / 373

页数：5

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