Angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma
Synonyms	immunoblastic lymphadenopathy (Lukes-Collins Classification), AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification)
Classification and external resources
Specialty	Hematology and oncology
ICD-10	C86.5 (ILDS C84.460)
ICD-O	9705/3
MeSH	D007119
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Angioimmunoblastic T-cell lymphoma (AITL, sometimes misspelled AILT) (formerly known as "Angioimmunoblastic lymphadenopathy with dysproteinemia") 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.

Patients with this disease usually present at an advanced stage and show systemic involvement. The clinical findings typically include a pruritic skin rash and possibly edema, ascites, pleural effusions, and arthritis.

Due to the systemic nature of this disease, neoplastic cells can be found in lymph nodes, liver, spleen, skin, and bone marrow.

This disease was originally thought to be a premalignant condition, termed angioimmunoblastic lymphadenopathy, and this atypical reactive lymphadenopathy carried a risk for transformation into a lymphoma. Currently, it is postulated that the originating cell for this disease is a mature (post-thymic) CD4+ T-cell that arises de novo, although some researchers argue that there is a premalignant subtype of this disease. The Epstein–Barr virus (EBV) is observed in the majority of cases, and the virus has been found in the reactive B-cells that comprise part of the polymorphous infiltrate of this disease and in the neoplastic T-cells. Immunodeficiency is also seen with this disease, but it is a sequela to the condition and not a predisposing factor.

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