Successful Treatment of Primary Amyloidosis: The authors reply

Abstract

We appreciate the comments of Dr. Manoharan. We would, however, caution against concluding that his two cases represent resolution of systemic amyloidosis in response to therapy with alkylating agents. In both patients, an objective decrease in production of monoclonal protein was demonstrated. This finding, however, cannot be assumed to be synonymous with the resolution of amyloid substance.1Kyle RA Wagoner RD Holley KE Primary systemic amyloidosis: resolution of the nephrotic syndrome with melphalan and prednisone.Arch Intern Med. 1982; 142: 1445-1447Crossref PubMed Scopus (67) Google Scholar In addition, the clinical manifestation of amyloidosis in his patients was carpal tunnel syndrome. In both patients, surgical release was performed. We have found that surgical intervention alone for carpal tunnel syndrome produces relief of symptoms, obviating the need for any further therapy. Finally, in the two references cited by Dr. Manoharan on the natural history and treatment of primary amyloidosis, no patient with only carpal tunnel syndrome was included. Patients with carpal tunnel syndrome must have additional serious organ involvement with amyloid to be considered as having a “systemic” condition and in need of potentially toxic treatment. We caution against the use of alkylating agents if the only manifestation of amyloidosis is carpal tunnel syndrome. Successful Treatment of Primary AmyloidosisMayo Clinic ProceedingsVol. 61Issue 10PreviewI read with interest the report by Drs. Gertz and Kyle in the March 1986 issue of the Proceedings (pages 218 through 223) in which regression of biopsy-proven hepatic amyloidosis was substantiated after treatment with melphalan and prednisone. Although my own experience with use of chemotherapy in three patients with primary hepatic amyloidosis and two other patients with primary cardiac amyloidosis has been disappointing, I have seen excellent responses in two patients with primary amyloidosis and carpal tunnel syndrome. Full-Text PDF