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Spectrum of lymph node pathology in adult onset Still's disease; analysis of 12 patients with one follow up biopsy
- Source :
- Journal of clinical pathology. 57(10)
- Publication Year :
- 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.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Herpesvirus 4, Human
Adolescent
T-Lymphocytes
Gene Rearrangement, B-Lymphocyte, Heavy Chain
Biology
Polymerase Chain Reaction
Pathology and Forensic Medicine
Pathogenesis
Biopsy
medicine
Humans
music
Lymph node
Antigens, Viral
Histiocyte
In Situ Hybridization
B-Lymphocytes
music.instrument
medicine.diagnostic_test
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
Anatomical pathology
General Medicine
Gene rearrangement
Original Articles
Hyperplasia
medicine.disease
Follicular hyperplasia
Immunohistochemistry
medicine.anatomical_structure
RNA, Viral
Female
Lymph Nodes
Still's Disease, Adult-Onset
Subjects
Details
- ISSN :
- 00219746
- Volume :
- 57
- Issue :
- 10
- Database :
- OpenAIRE
- Journal :
- Journal of clinical pathology
- Accession number :
- edsair.doi.dedup.....77423da7bf28932a7dcb88d85ecfcd3b