ABSTRACT
Fifty-one of 146 patients with primary amyloid deposits of light-chain origin (AL) examined between 1972 and 1986 were found to have peripheral neuropathy as part of their systemic amyloidosis. Peripheral neuropathy was the presenting symptom in 11 patients, and it was a later symptom or an incidental physical examination finding at the time of amyloid workup in 40 additional patients. The median duration of time from first symptom to the diagnosis of primary amyloidosis was longer in those whose first symptom was neuropathy (48 months vs 12 months). In addition, survival time after diagnosis for these patients was significantly longer (at least 35 months vs 16 months). Peripheral neuropathy is not an uncommon symptom of primary amyloidosis and may mark the onset of the disease process years before infiltration of vital structures of the heart, kidneys or gastrointestinal tract leads to failure and a subsequent downhill course. Appreciating amyloid disease at this early stage may widen the potential therapeutic window.
Subject(s)
Amyloidosis/complications , Peripheral Nervous System Diseases/etiology , Amyloidosis/mortality , Cardiovascular Diseases/etiology , Female , Gastrointestinal Diseases/etiology , Humans , Kidney Diseases/etiology , Male , Paresthesia/etiology , Peripheral Nervous System Diseases/epidemiology , Time FactorsABSTRACT
Samples of abdominal fat aspirates from 73 patients were sent to us for staining and interpretation. Ten samples were positive for amyloid. We calculated the sensitivity, specificity, and predictive value of the procedure based on the findings and the clinical information and other biopsy data about these patients. Using the results from more traditional biopsies as the "gold standard," sensitivity was 57%, specificity was 100%, and the predictive value was 100% for positive findings in the abdominal fat aspirate. Although the sample size in this study was relatively small, the procedure was found to be a minimally invasive test of high clinical utility.
Subject(s)
Adipose Tissue/analysis , Amyloid/analysis , Amyloidosis/diagnosis , Biopsy, Needle , Abdomen , Adult , Aged , Aged, 80 and over , Amyloidosis/metabolism , Female , Humans , Male , Middle Aged , Predictive Value of Tests , Prospective StudiesABSTRACT
Abdominal fat aspiration samples from 443 consecutive patients were examined for amyloid after Congo red and hematoxylin staining. Of the aspirates from 83 patients known to have systemic amyloid disease prior to the biopsy, 70 (84 percent) were found to yield positive results. The results for four aspirates from patients with localized amyloid disease were negative. Of the aspirates from 356 patients of unknown clinical status referred for analysis by outside physicians, 26 (7 percent) yielded positive results for amyloid. On review of the clinical records of these 26 patients, 11 had proved systemic amyloidosis demonstrated on biopsy of another site; all had a clinical course consistent with amyloid disease. In no case was amyloid found in a fat aspiration sample from a patient without clinical evidence suggestive of systemic amyloid disease. This study supports the proposal that abdominal fat aspiration is the diagnostic procedure of choice in the evaluation of amyloidosis since it requires no specialty consultation or technical expertise, causes minimal patient discomfort, and is accompanied by virtually no risk of morbid complication. A positive result has a high predictive value of amyloid disease in patients of unknown clinical status.
