ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (8)
Angioimmunoblastic lymphadenopathy
Other Resources UpToDate PubMed

Angioimmunoblastic lymphadenopathy

Contributors: Nina Haghi MD, Susan Burgin MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

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.

Codes

ICD10CM:
C86.5 – Angioimmunoblastic T-cell lymphoma

SNOMEDCT:
52097008 – Angioimmunoblastic lymphadenopathy

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

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:02/27/2020
Last Updated:02/27/2020
Copyright © 2021 VisualDx®. All rights reserved.
Angioimmunoblastic lymphadenopathy
Angioimmunoblastic lymphadenopathy : Fever, Hepatosplenomegaly, Night sweats, Hemolytic anemia, Hypergammaglobulinemia, LDH elevated, Lymphadenopathy, Urticaria, WBC elevated, EOS increased, Morbilliform rash
Clinical image of Angioimmunoblastic lymphadenopathy
Widespread brown and violaceous, scaly plaques on the chest and arm.
Copyright © 2021 VisualDx®. All rights reserved.