|
|
 Previous Article | Table of Contents | Next Article 
Response rates and survival in primary systemic amyloidosis
MA Gertz, RA Kyle and PR Greipp
Dysproteinemia Clinic, Mayo Clinic, Rochester, MN 55905.
Patients (153) with biopsy-proven primary systemic amyloidosis (AL) were
evaluated for their response rate to alkylating agent-based chemotherapy.
Twenty-seven of the patients (18%) responded. The serum creatinine
concentration had an adverse effect on response rate (P = .05). In patients
with nephrotic syndrome, a normal serum creatinine value, and no
echocardiographic evidence of cardiac amyloidosis, the response rate was
39% (12 of 31). Five of 34 patients with amyloid cardiomyopathy responded.
Two of these five are alive 10 years after diagnosis. None of the 18
patients with amyloid peripheral neuropathy showed regression of their
disease. The median time to achieve response was 11.7 months. The median
survival of the 27 patients was 89.4 months and 21 of 27 survived 5 years
(78%). Eight patients remain alive with a minimum follow-up of 90 months.
Seven died of acute leukemia or dysmyelopoietic syndrome, a presumed
complication of melphalan therapy. In the group of 126 patients who showed
no response to alkylating agent- based therapy, the median survival was
14.7 months and 9 (7%) survived over 5 years. All 126 patients have died.
Alkylating agent-based chemotherapy for AL is beneficial in a subset of
patients and a trial of chemotherapy is strongly recommended. Those
patients who do respond demonstrate survival benefit.
Volume 77,
Issue 2,
pp. 257-262,
01/15/1991
Copyright © 1991 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. D. Wechalekar, H. J. B. Goodman, H. J. Lachmann, M. Offer, P. N. Hawkins, and J. D. Gillmore
Safety and efficacy of risk-adapted cyclophosphamide, thalidomide, and dexamethasone in systemic AL amyloidosis
Blood,
January 15, 2007;
109(2):
457 - 464.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Dispenzieri, M. Q. Lacy, S. R. Zeldenrust, S. R. Hayman, S. K. Kumar, S. M. Geyer, J. A. Lust, J. B. Allred, T. E. Witzig, S. V. Rajkumar, et al.
The activity of lenalidomide with or without dexamethasone in patients with primary systemic amyloidosis
Blood,
January 15, 2007;
109(2):
465 - 470.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H J Lachmann and P N Hawkins
Amyloidosis and the lung
Chronic Respiratory Disease,
November 1, 2006;
3(4):
203 - 214.
[Abstract]
[PDF]
|
|
|
|
|
|
|
A. Dispenzieri, M. Q. Lacy, J. A. Katzmann, S. V. Rajkumar, R. S. Abraham, S. R. Hayman, S. K. Kumar, R. Clark, R. A. Kyle, M. R. Litzow, et al.
Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation
Blood,
April 15, 2006;
107(8):
3378 - 3383.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. D. Gillmore, H. J. Goodman, H. J. Lachmann, M. Offer, A. D. Wechalekar, J. Joshi, M. B. Pepys, and P. N. Hawkins
Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis
Blood,
February 1, 2006;
107(3):
1227 - 1229.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. F. Porrata, M. A. Gertz, M. R. Litzow, M. Q. Lacy, A. Dispenzieri, D. J. Inwards, S. M. Ansell, I. N.M. Micallef, D. A. Gastineau, M. Elliott, et al.
Early Lymphocyte Recovery Predicts Superior Survival after Autologous Hematopoietic Stem Cell Transplantation for Patients with Primary Systemic Amyloidosis
Clin. Cancer Res.,
February 1, 2005;
11(3):
1210 - 1218.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. V. Dhodapkar, M. A. Hussein, E. Rasmussen, A. Solomon, R. A. Larson, J. J. Crowley, and B. Barlogie
Clinical efficacy of high-dose dexamethasone with maintenance dexamethasone/alpha interferon in patients with primary systemic amyloidosis: results of United States Intergroup Trial Southwest Oncology Group (SWOG) S9628
Blood,
December 1, 2004;
104(12):
3520 - 3526.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Dispenzieri, R. A. Kyle, M. Q. Lacy, T. M. Therneau, D. R. Larson, M. F. Plevak, S. V. Rajkumar, R. Fonseca, P. R. Greipp, T. E. Witzig, et al.
Superior survival in primary systemic amyloidosis patients undergoing peripheral blood stem cell transplantation: a case-control study
Blood,
May 15, 2004;
103(10):
3960 - 3963.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Kumar, T. K. Kimlinger, J. A. Lust, K. Donovan, and T. E. Witzig
Expression of CD52 on plasma cells in plasma cell proliferative disorders
Blood,
August 1, 2003;
102(3):
1075 - 1077.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D.S. Wald and H.H. Gray
Restrictive cardiomyopathy in systemic amyloidosis
QJM,
May 1, 2003;
96(5):
380 - 382.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Pardanani, T. E. Witzig, G. Schroeder, E. A. McElroy, R. Fonseca, A. Dispenzieri, M. Q. Lacy, J. A. Lust, R. A. Kyle, P. R. Greipp, et al.
Circulating peripheral blood plasma cells as a prognostic indicator in patients with primary systemic amyloidosis
Blood,
February 1, 2003;
101(3):
827 - 830.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. A. Gertz
Diagnosing Primary Amyloidosis
Mayo Clin. Proc.,
December 1, 2002;
77(12):
1278 - 1279.
[PDF]
|
|
|
|
|
|
|
A. Dispenzieri, M. Q. Lacy, R. A. Kyle, T. M. Therneau, D. R. Larson, S. V. Rajkumar, R. Fonseca, P. R. Greipp, T. E. Witzig, J. A. Lust, et al.
Eligibility for Hematopoietic Stem-Cell Transplantation for Primary Systemic Amyloidosis Is a Favorable Prognostic Factor for Survival
J. Clin. Oncol.,
July 15, 2001;
19(14):
3350 - 3356.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S W Dubrey, M M Burke, A Khaghani, P N Hawkins, M H Yacoub, and N R Banner
Long term results of heart transplantation in patients with amyloid heart disease
Heart,
February 1, 2001;
85(2):
202 - 207.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
N. Iggo, T. Littlewood, and C.G. Winearls
Prospects for effective treatment of AL amyloidosis?
QJM,
May 1, 2000;
93(5):
257 - 260.
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. A. Gertz, M. Q. Lacy, J. A. Lust, P. R. Greipp, T. E. Witzig, and R. A. Kyle
Prospective Randomized Trial of Melphalan and Prednisone Versus Vincristine, Carmustine, Melphalan, Cyclophosphamide, and Prednisone in the Treatment of Primary Systemic Amyloidosis
J. Clin. Oncol.,
January 1, 1999;
17(1):
262 - 262.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. L. Comenzo, E. Vosburgh, R. H. Falk, V. Sanchorawala, J. Reisinger, S. Dubrey, L. M. Dember, J. L. Berk, G. Akpek, M. LaValley, et al.
Dose-Intensive Melphalan With Blood Stem-Cell Support for the Treatment of AL (Amyloid Light-Chain) Amyloidosis: Survival and Responses in 25 Patients
Blood,
May 15, 1998;
91(10):
3662 - 3670.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L B Lovat, M R Persey, S Madhoo, M B Pepys, and P N Hawkins
The liver in systemic amyloidosis: insights from 123I serum amyloid P component scintigraphy in 484 patients
Gut,
May 1, 1998;
42(5):
727 - 734.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Dhodapkar, B. Barlogie, M. Gertz, D. R. Jacobson, G. Gallo, J. N. Buxbaum, S. Mahmoud, R. H. Falk, R. L. Comenzo, and M. Skinner
The Systemic Amyloidoses
N. Engl. J. Med.,
January 22, 1998;
338(4):
264 - 265.
[Full Text]
|
|
|
|
|
|
|
P. N HAWKINS
Diagnosis and treatment of amyloidosis
Ann Rheum Dis,
November 1, 1997;
56(11):
631 - 633.
[Full Text]
|
|
|
|
|
|
|
R. H. Falk, R. L. Comenzo, and M. Skinner
The Systemic Amyloidoses
N. Engl. J. Med.,
September 25, 1997;
337(13):
898 - 909.
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. S. Kushwaha, J. T. Fallon, and V. Fuster
Restrictive Cardiomyopathy
N. Engl. J. Med.,
January 23, 1997;
336(4):
267 - 276.
[Full Text]
[PDF]
|
|
|
|
|
|
|
I. Kronzon, M. Fedor, D. Schwartz, G. Gallo, and D. R. Jacobson
A 58-Year-Old Man With Shortness of Breath, Ascites, and Leg Edema
Circulation,
September 15, 1996;
94(6):
1483 - 1488.
[Full Text]
|
|
|
|
|
|
|
R Hall and P N Hawkins
Grand Rounds - Hammersmith Hospital: Cardiac transplantation for AL amyloidosis
BMJ,
October 29, 1994;
309(6962):
1135 - 1137.
[Full Text]
|
|
|
|
|
