How I Diagnose Angioimmunoblastic T-Cell Lymphoma

Yi Xie, MD, PhD; Elaine S. Jaffe, MD

Disclosures

Am J Clin Pathol. 2021;156(1):1-14. 

In This Article

Clinical Features

AITL primarily affects middle-aged and elderly adults, with a median age in the fifth to sixth decades.[16] There is a slight male predominance.[17] Most patients have advanced-stage disease (stages III-IV). Extranodal involvement is common. Characteristic features at presentation include constitutional symptoms, generalized lymphadenopathy (typically <1–3 cm), hepatosplenomegaly, and skin rash.[18] A significant proportion of patients also have pleural effusions and/or ascites. Bone marrow involvement has been reported in up to 70% of the cases.[19] Involvement of other extranodal sites such as lung and gastrointestinal tract can be seen but is less common.[20,21]

In addition to lymphoma-associated symptoms, many patients have immune dysregulation, resulting in autoimmune complications and immunodeficiency. There is usually polyclonal hypergammaglobulinemia and Coombs-positive hemolytic anemia.[22] Polyarthritis, vasculitis, and thyroid abnormalities are common. Laboratory tests frequently reveal circulating immune complexes, autoantibodies, cold agglutinins, elevated LDH, and hematologic abnormalities such as anemia, lymphopenia, thrombocytopenia, or eosinophilia.[23] Some patients may show evidence of immunodeficiency with opportunistic infections.[24] AITL may have a waxing and waning course, but it is generally aggressive, with a median survival of 3 years and a 5-year overall survival rate of approximately 30%.[23]

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