Angioimmunoblastic T-cell lymphoma
Angioimmunoblastic T-cell lymphoma | |
---|---|
Other names | immunoblastic lymphadenopathy (Lukes-Collins Classification), AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification)[1] |
Specialty | Hematology and oncology |
Angioimmunoblastic T-cell lymphoma (AITL, sometimes misspelled AILT, formerly known as "angioimmunoblastic lymphadenopathy with dysproteinemia"[2]: 747 ) is a mature T-cell lymphoma of blood or lymph vessel immunoblasts characterized by a polymorphous lymph node infiltrate showing a marked increase in follicular dendritic cells (FDCs) and high endothelial venules (HEVs) and systemic involvement.[1]
Signs and symptoms
Patients with AITL usually present at an advanced stage and show
Sites of involvement
Due to the systemic nature of AITL, neoplastic cells can be found in
]Causes
AITL was originally thought to be a premalignant condition, termed angioimmunoblastic lymphadenopathy, and this atypical reactive lymphadenopathy carried a risk for transformation into a lymphoma. It is postulated that the originating cell for AITL is a mature (post-thymic) CD4+
Diagnosis
Laboratory findings
The classical laboratory finding is polyclonal
Lymph node
The normal architecture of a lymph node is partially effaced by a polymorphous infiltrate and residual follicles are commonly seen. The polymorphous infiltrate consists of lymphocytes of moderate size with pale/clear cytoplasm and smaller reactive
Immunophenotype
AITL typically has the phenotype of a mixture of
Molecular findings
Clonal
Treatment
There is no proven or standard first-line chemotherapy that works for the majority of AITL patients. There are several
Epidemiology
The typical patient with angioimmunoblastic T-cell lymphoma (AITL) is either middle-aged or elderly, and no gender preference for this disease has been observed.[1] AITL comprises 15–20% of peripheral T-cell lymphomas and 1–2% of all non-Hodgkin lymphomas.[16]