Clinical study of 31 cases of primary amyloidosis
Pigrau, C.; Martínez-Vázquez, J.M.; Ocaña, I.; Muñiz, R.; Capdevila, J.A.; Ribera, E.; Lorente, A.; Vidal, M.T.; Moragas, A.
Medicina Clinica 88(10): 399-404
1987
ISSN/ISBN: 0025-7753 PMID: 3573837 Document Number: 305300
Thirty-one patients with primary amyloidosis have been retrospectively studied (25 idiopathic, 5 associated with myeloma and 1 to Waldenstrom's disease). Histological diagnosis had been made during a 14-year period. The major presenting symptoms were ankle edema, weight loss and dyspnea. Organ distributrion was as follows: renal in 83% (25/31), gastrointestinal in 67% (21/31), cardiac in 61% (19/31), and neuropathic in 19% (6/31). The high prevalence of adrenal involvement (50%) in 10 cases studied at necropsy is remarkable. A homogeneous component in plasma and/or urine was present in 95% of patients (19/20). 64% (16/25) of patients died; the major causes of mortality were sudden death, sepsis and heart failure.