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.
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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% 3.
History and etymology
It was initially described in 1974 by Frizzera et al as angioimmunoblastic lymphadenopathy with dysproteinemia (AILD).
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