Angioimmunoblastic lymphadenopathy
Alerts and Notices
Synopsis

Angioimmunoblastic T-cell lymphoma (AITL) is one of the more common peripheral T-cell lymphomas. AITL represents approximately 1%-2% of non-Hodgkin lymphomas and approximately 20% of peripheral T-cell lymphomas. The median age of diagnosis is 65 years. AITL is thought to arise from follicular helper T-cells.
Patients often present with acute onset of symptoms with lymphadenopathy, fevers, unintentional weight loss, night sweats, and hepatomegaly or splenomegaly. Approximately 20%-50% of patients present with cutaneous findings, including an exanthematous eruption, urticaria, and infiltrated papules and nodules. Generalized pruritus may be an early symptom. AITL can mimic an immune system disorder with many similar signs such as joint pain, swelling, ascites, pleural effusion, and/or hemolytic anemia. It is often difficult to diagnose for this reason. Lymph node biopsy demonstrates a polymorphous infiltrate with proliferation of follicular dendritic cells and high endothelial venules.
Prognosis of AITL is often poor with a high mortality rate of 50%-75%. The 5-year median survival rate is 32%, which varies depending on certain prognostic factors.
Patients often present with acute onset of symptoms with lymphadenopathy, fevers, unintentional weight loss, night sweats, and hepatomegaly or splenomegaly. Approximately 20%-50% of patients present with cutaneous findings, including an exanthematous eruption, urticaria, and infiltrated papules and nodules. Generalized pruritus may be an early symptom. AITL can mimic an immune system disorder with many similar signs such as joint pain, swelling, ascites, pleural effusion, and/or hemolytic anemia. It is often difficult to diagnose for this reason. Lymph node biopsy demonstrates a polymorphous infiltrate with proliferation of follicular dendritic cells and high endothelial venules.
Prognosis of AITL is often poor with a high mortality rate of 50%-75%. The 5-year median survival rate is 32%, which varies depending on certain prognostic factors.
Codes
ICD10CM:
C86.5 – Angioimmunoblastic T-cell lymphoma
SNOMEDCT:
52097008 – Angioimmunoblastic lymphadenopathy
C86.5 – Angioimmunoblastic T-cell lymphoma
SNOMEDCT:
52097008 – Angioimmunoblastic lymphadenopathy
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
- Other B-cell or T-cell lymphomas
- Hodgkin lymphoma
- Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma
- Autoimmune disease
- Castleman disease
- Infection (viral lymphadenitis, especially infectious mononucleosis)
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
Subscription Required
References
Subscription Required
Last Reviewed:02/26/2020
Last Updated:02/26/2020
Last Updated:02/26/2020