Angioimmunoblastic T cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) (previously known as angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)) is a rare, aggressive (fast-growing) form of peripheral T-cell lymphoma. It only accounts for around 1-2% of all non-Hodgkin lymphoma. It is one of the more common subtypes of mature T-cell lymphomas.

Epidemiology

It is more common in the elderly.

Clinical presentation

The clinical spectrum includes:

• high fevers
• generalized lymphadenopathy
• skin rash
• hepatosplenomegaly
• hemolytic anemia 

Treatment and prognosis

Despite advances in treatment regimens for angioimmunoblastic T cell lymphoma, prognosis remains poor with an overall 5-year median survival of 32% .

History and etymology

It was initially described in 1974 by Frizzera et al as angioimmunoblastic lymphadenopathy with dysproteinemia (AILD).