• Thirty-two patients with primary amyloidosis (AL) either due to multiple myeloma or without apparent cause were studied. The most common manifestations were carpal tunnel syndrome and nephrosis. Rectal biopsy specimens showed amyloid infiltrates in 65% (15 of 23) of the patients studied, but virtually all biopsies of normal skin were negative. λ Light chains were present in 75% (21 of 28) of patients with a monoclonal gammopathy. The median survival time was 14 months after histologic diagnosis, following a median delay of seven months from the recognition of disease by a physician. An earlier diagnosis would have been possible in most of the patients if the initial findings of amyloidosis had been recognized and evaluated definitively. The prognosis remains poor, because effective therapy Is not available.
(Arch Intern Med 1984;144:2158-2160)