Metastatic angioimmunoblastic T-cell lymphoma started from thoracic paravertebral region: a Case report

Angioimmunoblastic T-cell lymphoma (AITL) is one of the most frequent nodal T-cell lymphoma. 1,2 It derives from follicular helper T-cell (TFH).3 It accounts for 15 - 20% of all peripheral T-cell lymphomas and usually affects patients in the seventh decade of life.1,2,4,5 AITL’s incidence is nearly 0,05 new patient case per 100,000 people in US, and there’s no sex predilection.6,7 It is characterized by polymorphic lymph node infiltrate with a prominent proliferation of high endothelial venules and follicular dendritic cells, different immune disorders and a poor prognosis. 8,9 The neoplastic T-cells express CD2, CD3, CD4 and CD10 but the marker’s specificity has been debated. More specific indicators of AITL are CXCL-13, programmed death-1 (PD1), inducible costimulator (ICOS), and BCL6 transcription factor.10-12 Nearly all patients have EBV-infected B cells in their lymph nodes, but the presence of these EBV-positive cells doesn’t correlate with survival.13-15 However, the role of EBV isn’t clear yet: it could be secondary to the immune deregulation, or it could be a fundamental factor involved in disease’s start and progression. AITL is frequently associated with polyclonal B-cell or plasma cell proliferation;8 this neoplastic proliferation of B-cells on parallel with AITL could be motivated by a cluster of pluripotent cells with the ability to differentiate into B-cells and T-cells neoplasm simultaneously, maybe due to exposition to pharmacological therapèies or specific mutagens. Clinical manifestations are often represented by group-B symptoms (fever, night sweats, weight loss), hepatosplenomegaly, anemia, lymphadenopathy, polyclonal hypergammaglobulinemia, thrombocytopenia and/or a large variety of immune disorders.16,17 Up to 50% of develop cutaneous lesions, expression of extranodal diffusion of the tumor: urticaria, purpura, pruritic maculopapular eruptions, erosions, plaques, nodules, petechiae.18-20 Despite occasionally spontaneous remissions,21 AITL prognosis is poor, with a median overall survival of 3 years.