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Cutaneous Infarcts and Antineutrophil Cytoplasmic Antibody‑Positive Vasculitis in Angioimmunoblastic T‑Cell Lymphoma: A Rare PresentationFinancial support and sponsorship Nil.
Angioimmunoblastic T-cell lymphoma (AITL) is a rare and unique subtype of peripheral T-cell lymphoma, accounting for 2%–5% of all non-Hodgkin’s lymphomas. The lymph node architecture is often effaced, with retention of only a few benign follicles. The infiltrate characteristically extends beyond the lymph node capsule, with preserved and dilated subcapsular sinus. Often, there is interfollicular proliferation of endothelial venules. The neoplastic T-cells are positive for CD2, CD3, CD4, CD10, CXCL-13, PD1, and often BCL-6.
AITL is associated with many systemic features such as fever, rash, or arthritis. These systemic features may precede the appearance of other features of lymphoma. The disease may present as autoimmune phenomena, such as the presence of circulating immune complexes, cold agglutinins, hemolytic anemia, and rheumatoid factor and anti-smooth muscle antibodies. Hypergammaglobulinemia is present in approximately 50% of patients, typically polyclonal. Vasculitis is occasionally associated with AITL, but it is usually antineutrophil cytoplasmic antibody (ANCA)-negative vasculitis.
Received: 14 June 2018
Accepted: 12 July 2019
Article published online:
03 June 2021
© 2020. Indian Society of Medical and Paediatric Oncology. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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