Rosai-Dorfman disease and generalized AA amyloidosis: a case report.
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.
Röcken
C
C
Institute of Pathology and Department of Haematology and Oncology, Otto-von-Guericke-University, Magdeburg, Germany.
Wieker
K
K
Grote
H J
HJ
Müller
G
G
Franke
A
A
Roessner
A
A
D000328:Adult; D000686:Amyloidosis / Q000150:complications* / Q000378:metabolism* / Q000473:pathology; D002097:C-Reactive Protein / Q000032:analysis; D017809:Fatal Outcome; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D000685:Serum Amyloid A Protein / Q000378:metabolism*
D000685:Serum Amyloid A Protein; D002097:C-Reactive Protein