CC BY-NC-ND 4.0 · Indian J Med Paediatr Oncol 2019; 40(04): 598-600
DOI: 10.4103/ijmpo.ijmpo_133_18
Letter to Editor

Cutaneous Infarcts and Antineutrophil Cytoplasmic Antibody‑Positive Vasculitis in Angioimmunoblastic T‑Cell Lymphoma: A Rare Presentation

Ashwin Mahesh
Department of General Medicine, Army College of Medical Sciences, Delhi Cantt., New Delhi
Sandeep Kumar
Department of General Medicine, Army College of Medical Sciences, Delhi Cantt., New Delhi
Abhish Mohan
Department of General Medicine, Armed Forces Medical College, Pune, Maharashtra
Niket Verma
Department of General Medicine, Army College of Medical Sciences, Delhi Cantt., New Delhi
› Author Affiliations
Financial 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.[1] 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.[2]

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.[3] Vasculitis is occasionally associated with AITL, but it is usually antineutrophil cytoplasmic antibody (ANCA)-negative vasculitis.[4]

Publication History

Received: 14 June 2018

Accepted: 12 July 2019

Article published online:
03 June 2021

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  • References

  • 1 Rüdiger T, Weisenburger DD, Anderson JR, Armitage JO, Diebold J, MacLennan KA. et al. Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): Results from the non-Hodgkin’s lymphoma classification project. Ann Oncol 2002; 13: 140-9
  • 2 Attygalle AD, Diss TC, Munson P, Isaacson PG, Du MQ, Dogan A. CD10 expression in extranodal dissemination of angioimmunoblastic T-cell lymphoma. Am J Surg Pathol 2004; 28: 54-61
  • 3 Frizzera G, Moran EM, Rappaport H. Angio-immunoblastic lymphadenopathy with dysproteinaemia. Lancet 1974; 1: 1070-3
  • 4 Hamidou MA, El Kouri D, Audrain M, Grolleau JY. Systemic antineutrophil cytoplasmic antibody vasculitis associated with lymphoid neoplasia. Ann Rheum Dis 2001; 60: 293-5
  • 5 Iannitto E, Ferreri AJ, Minardi V, Tripodo C, Kreipe HH. Angioimmunoblastic T-cell lymphoma. Crit Rev Oncol Hematol 2008; 68: 264-71