Primary systemic amyloidosis--a diagnostic primer.
Primary systemic amyloidosis (immunoglobulin light chain-derived) (AL) is an uncommon dysproteinemia with highly varied initial clinical manifestations. Among 153 patients with this disorder, the median survival was 20.4 months (5-year survival, 19.6%). The worst outcome was associated with overt congestive heart failure (median survival, 7.7 months; 5-year survival, 2.4%). The patients with the best outcome were those who had amyloid neuropathy without associated cardiac or renal involvement (median survival, 39.7 months; 5-year survival, 31.6%). Serum protein electrophoresis and immunoelectrophoresis are the most important tests because a monoclonal protein can be detected in almost two-thirds of the patients. When screening of both serum and urine is performed, a monoclonal protein is found in 86% of patients. Such screening is helpful if primary systemic amyloidosis is to be detected and treated early.
Gertz
M A
MA
Dysproteinemia Clinic, Mayo Clinic, Rochester, MN 55905.
Kyle
R A
RA
D000686:Amyloidosis / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D002311:Cardiomyopathy, Dilated / Q000209:etiology; D002349:Carpal Tunnel Syndrome / Q000150:complications; D003937:Diagnosis, Differential; D004586:Electrophoresis; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis; D008875:Middle Aged; D009101:Multiple Myeloma / Q000097:blood / Q000175:diagnosis; D009404:Nephrotic Syndrome / Q000209:etiology; D010292:Paresthesia / Q000209:etiology; D011379:Prognosis; D000685:Serum Amyloid A Protein / Q000032:analysis; D012737:Sex Factors
D007147:Immunoglobulin Light Chains; D000685:Serum Amyloid A Protein