Angioimmunoblastic T-cell lymphoma

Background

Subtype of mature peripheral T-cell lymphoma
Previously also known as angioimmunoblastic lymphadenopathy with dysproteinemia, immunoblastic lymphadenopathy, and lymphogranulomatosis X
Accounts for 1 to 2% of non-Hodgkin lymphoma
Derives from follicular T helper (T_FH) cells

Myriad and often non-specific, resulting in delayed diagnoses
Usually advanced stage at diagnosis
Most common symptoms are constitutional symptoms (fevers, night sweats, weight loss) and lymphadenopathy
May have hepatosplenomegaly
May have skin lesions which range from urticaria to nodules
May get rash after antibiotics
Often have elevated LDH, anemia, positive DAT, hypergammaglobulinemia (polyclonal)
May have hypereosinophilia, occasionally have thrombocytopenia
May have false-positive autoimmune tests, including rheumatoid factor and anti-smooth muscle antibodies