Rosai-Dorfman disease and generalized AA amyloidosis: a case report

Hum Pathol. 2000 May;31(5):621-4. doi: 10.1053/hp.2000.6705.


We report on a patient who, at 31 years of age, was found to suffer from sinus histiocytosis with massive lymphadenopathy (SHML; Rosai-Dorfman disease) with nodal and extranodal involvement as described previously. Five years later the patient presented with nephrotic syndrome caused by a generalized AA amyloidosis, and he subsequently died from pulmonary thromboembolism owing to renal vein thrombosis. Retrospective analysis of serum levels of C-reactive protein (CRP) showed that during the last 3 years before his death, he had a persistently elevated CRP level ranging from 73 to 161 mg/L, despite antiinflammatory treatment with prednisolone, methotrexate, or 6-mercaptopurine. These figures indicate that the patient was probably suffering from a permanent acute phase response which, in the absence of any other evidence of a chronic inflammatory disease which commonly causes AA amyloidosis, was most likely owing to SHML.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Amyloidosis / complications*
  • Amyloidosis / metabolism*
  • Amyloidosis / pathology
  • C-Reactive Protein / analysis
  • Fatal Outcome
  • Histiocytosis, Sinus / complications*
  • Histiocytosis, Sinus / pathology
  • Humans
  • Male
  • Serum Amyloid A Protein / metabolism*


  • Serum Amyloid A Protein
  • C-Reactive Protein