Angioimmunoblastic T-cell lymphoma
Synonyms: AILT | Immunoblastic lymphadenopathy | Lymphogranulomatosis X | T-cell lymphoma, AILD type
A rare T-cell non-Hodgkin lymphoma characterized by infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells as well as prominent proliferation of high endothelial venules. The spleen liver skin and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy hepatosplenomegaly systemic symptoms and polyclonal hypergammaglobulinemia. Pruritic skin rash arthritis pleural effusion and ascites may also be observed. The condition is aggressive with generally poor prognosis.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
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Angioimmunoblastic T-cell lymphoma?
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