A Report of Three Cases

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ID: 296169
1986
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Abstract
The authors report the pathologic features of three cases of amyloidosis associated with cystic fibrosis. Renal biopsy led to the diagnosis (case 1) or suspicion (case 2) of amyloidosis in patients who were 23 and 21 years old, respectively. The third patient died at age 22 years, and amyloidosis was not discovered until autopsy. Immunohistochemical staining and potassium-permanganate pretreatment of histologic sections in all three cases provided evidence that the amyloid seen in these patients is of the secondary (AA) type. Congo red staining in each case and electron microscopy in case 1 confirmed the initial diagnosis of amyloidosis. A markedly elevated serum amyloid A protein (160 μg/mL; normal less than 1 μg/mL) in case 1 indicated the presence of large quantities of the precursor protein from which the AA fibrils of secondary amyloid are derived. The kidneys, spleen, and liver contained amyloid deposits in autopsy material from all three cases. Involvement of other organs by amyloid was variable. Review of autopsy material in Boston from 23 additional cystic fibrosis patients with long-term survival did not reveal any evidence of amyloidosis. It appears that secondary amyloidosis is emerging as a significant, although rare, complication of cystic fibrosis as greater numbers of these patients survive into adult-hood.
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openalex_W2396634286 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors William D. Travis, Robert G. Castile, Gordon F. Vawter, Harry Shwachman, Warren J. Warwick, Barbara Burke, Martha Skinner
Journal American Journal of Clinical Pathology
Year 1986
DOI
10.1093/ajcp/85.4.419
URL
https://doi.org/10.1093/ajcp/85.4.419
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