A difficult case of angioimmunoblastic T-cell lymphoma to diagnose

摘要

Angioimmunoblastic T-cell lymphoma (AITL) is a malignancy of mature T-cells. It is characterized as a polymorphic lymphonodal lymphoid infiltrate accompanied by prominent proliferation of endothelial venules and follicular dendritic cells. AITL was first described in 1974 by Frizzera et al. as an angioimmunoblastic lymphadenopathy with dysproteinemia.1 A short time later, the name was changed to immunoblastic lymphadenopathy, and then to lymphogranulomatosis X in 1979.2AITL comprises 1520% of all peripheral T-cell lymphomas and 12% of all non-Hodgkin lymphomas (NHL). Most frequently, it occurs in aged patients, with equal prevalence between males and females. Typically, AITL displays an aggressive behavior, which makes the diagnosis difficult and it must be differentiated from other malignant lymphoproliferative diseases, drug reactions and viral infections. Patients with AITL frequently exhibit B-symptoms (e.g., fever and weight loss) and a generalized enlargement of the lymph nodes. Other common symptoms include hepatomegaly, splenomegaly, polymorphic skin rash and pleural effusion. Advanced stage disease (Ann Arbor III/IV) is observed in 80% of cases. AITL is also associated with autoimmune phenomena. Polyclonal hypergammaglobulinemia occurs in approximately 50% of AITL cases.3.