Elsevier

Human Pathology

Volume 31, Issue 5, May 2000, Pages 621-624
Human Pathology

Case study
Rosai-Dorfman disease and generalized AA amyloidosis: A case report*

https://doi.org/10.1053/hp.2000.6705Get rights and content

Abstract

We report on a patient who, at 31 years of age, was found tosuffer 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 anti-inflammatory 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 SHAM.

References (15)

There are more references available in the full text version of this article.

Cited by (31)

  • Rosai-Dorfman Disease Presenting as Peripheral Vascular Insufficiency

    2018, Annals of Vascular Surgery
    Citation Excerpt :

    In some cases, however, the syndrome assumes more severe and extensive clinical characteristics, which require surgical treatment to reduce disease progression.29 Prognosis is generally influenced by the number of lymphatic stations and the extra lymphatic sites involved by the disease, and the possible involvement of heart, liver, kidneys, upper airways, skeleton (with osteolytic lesions), and CNS; there are known reported cases of acute renal insufficiency30,31 and linfomas.32 In these last years, there has been an increased understanding of the biology of these disorders, along with potential for adequate therapy.

  • Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease

    2018, Blood
    Citation Excerpt :

    The kidneys are affected in 4% of RDD cases, with a discrete mass or diffuse infiltration.49,50 Symptoms include hematuria, flank pain, abdominal fullness, renal failure, hypercalcemia, or nephrotic syndrome caused by amyloidosis or renal vein thrombosis.51-54 Hydronephrosis and ureteral obstruction can occur.55

  • Rosai-Dorfman disease

    2007, Presse Medicale
  • Advanced glycation end products and receptor for advanced glycation end products in AA amyloidosis

    2003, American Journal of Pathology
    Citation Excerpt :

    Variable amounts of amyloid were present in all of the specimens as vascular and interstitial deposits. In addition, unfixed splenic tissue containing amyloid was available from three of the above-mentioned patients with generalized AA amyloidosis14,24 and this was used for the preparation of amyloid fibril proteins as described previously25 using ∼6 g of amyloidotic tissue.24 Six- to 8-week-old BALB/c female mice were purchased from Harlan Winkelmann GmbH (Borchen, Germany).

View all citing articles on Scopus
*

This work was supported by grants from the Deutsche Forschungsgemeinschaft,Bonn-Bad Godesberg, Grant No. Ro 1173/2-1.

View full text