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

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 c chain (TCRc) 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 TCRc 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.

Adulter Morbus Still, Fieber, Diagnose und Therapie

“…Eine Lymphadenopathie findet sich bei 50-70% der AOSD-Patienten [15]. Die Lymphadenopathie tritt zwar gehäuft bei AOSD-Patienten auf.…”

Adult - onsetStill′s disease: A review

“…It occurs worldwide and has been reported from France, [5,6] Norway, [7] Japan, [8,9] Greece, [10] USA, [11] Taiwan, [12,13] Spain, [14] Korea, [15,16] China, [17,18] Turkey, [19,20] India [21,22] and Iran. [23] The disease affects young people and has a bimodal age distribution with two peaks -at 15-25 and 36-46 years of age.…”

“…Occasionally, lymph node histology in AOSD may mimic malignant lymphoma. [16] Hepatosplenomegaly is common in early disease and reß ects tissue inÞ ltration by inflammatory cells. [19,23,80] Macrophage activation syndrome (MAS) is a lifethreatening condition, which is characterized by uncontrolled activation and proliferation of T lymphocytes and macrophages in bone marrow, reticuloendothelial system and central nervous system.…”

Adult-onset Still's disease

“…Кроме того, ИЛ 1 отводят важ-ную роль в проявлениях болезни, нарастании циркули-рующих нейтрофилов, индукции лихорадки и регуляции уровня ферритина [43]. Потенциальная роль вовлечения В-клеток в патогенез БСРВ подтверждается гистологиче-скими и иммуногистохимическими результатами иссле-дования биоптатов лимфатических узлов, хотя это требу-ет уточнения [44]. Установлено, что помимо продукции антител В-клетки действуют как антиген-презентирую-щие клетки, активируя Т-клетки, а недавно им стали от-водить важную роль в регуляции синтеза провоспали-тельных цитокинов [45].…”