View source for 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<sub>FH</sub>) cells

== Clinical Manifestations ==

* 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

=== Prognosis ===

* 5-year survival about 30%

== Further Reading ==

* Angioimmunoblastic T-cell lymphoma: the many-faced lymphoma. ''Blood''. 2017;129(9):1095-1102. doi: [https://doi.org/10.1182/blood-2016-09-692541 10.1182/blood-2016-09-692541]

[[Category:Hematology]]
[[Category:Oncology]]