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J Clin Pathol 2004;57:1052-1056 doi:10.1136/jcp.2004.018010
  • Original article

Spectrum of lymph node pathology in adult onset Still’s disease; analysis of 12 patients with one follow up biopsy

  1. Y K Jeon1,
  2. J H Paik1,
  3. S-S Park1,
  4. S O Park1,
  5. Y A Kim2,
  6. J E Kim2,
  7. Y W Song3,
  8. C W Kim1
  1. 1Department of Pathology and Cancer Research Institute, Tumour Immunity Medical Research Centre, Seoul National University College of Medicine, Seoul 110–799, Korea
  2. 2Department of Pathology, Seoul City Boramae Hospital, Seoul 156–707, Korea
  3. 3Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine
  1. Correspondence to:
 Dr C W Kim
 Department of Pathology, Seoul National University College of Medicine, 28 Yongon-dong, Chongno-gu, Seoul, 110–799, Korea; cwkimplaza.snu.ac.kr
  • Accepted 1 May 2004

Abstract

Background: Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology, frequently accompanying multiple lymphadenopathy. It often mimics malignant lymphoma, and immunohistochemical and molecular studies are needed for definite diagnosis.

Aims: To aid in diagnosis and understand the pathogenesis of the disease by clarifying lymph node (LN) pathology in AOSD.

Methods: Thirteen biopsies (one follow up biopsy) and medical records of 12 patients were reviewed. Immunohistochemistry, polymerase chain reaction for T cell receptor γ chain (TCRγ) and immunoglobulin heavy chain gene rearrangement, and Epstein-Barr virus in situ hybridisation were performed.

Results: Histologically, LN lesions were classified into four patterns. The most common (six biopsies) showed paracortical hyperplasia, with prominent vascular proliferation, scattered large B/T immunoblasts, and infiltration by reactive lymphocytes and inflammatory cells. In the second pattern (two biopsies), paracortical hyperplasia was accompanied by massive sinus histiocytosis and S-100 positive histiocyte aggregates. The third pattern (three patients) showed an exuberant immunoblastic reaction, in the form of patchy/diffuse infiltration of large T immunoblasts with high mitotic activity, although clonal rearrangement of the TCRγ gene was not detected. The fourth pattern showed distinct follicular hyperplasia (two cases). One patient with a follow up biopsy showed a pattern change from pronounced follicular hyperplasia to atypical paracortical hyperplasia.

Conclusions: AOSD LN lesions show a dynamic histological spectrum, including atypical paracortical hyperplasia, burnt out histiocytic reaction, exuberant immunoblastic reaction, and follicular hyperplasia. During the course of disease, LN reactivity changes and mixed B and T cells are involved in the pathogenesis.

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