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Angioimmunoblastic lymphadenopathy with dysproteinemia
Angioimmunoblastic lymphadenopathy with dysproteinemia [OBS]
Angioimmunoblastic lymphoma [OBS]
Peripheral T-cell lymphoma, AILD
Peripheral T-cell lymphoma characterized by systemic disease; a polymorphous infiltrate involving lymph nodes, with a prominent proliferation of high endothelial venules and follicular dendritic cells.
Angioimmunoblastic T-cell lymphoma (AITL) primarily involves the lymph nodes and virtually all patients present with generalized lymphadenopathy. In addition, spleen, liver, and bone marrow are frequently involved.
AITL typically presents with advanced stage disease, generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Skin rash, often with pruritis, is frequently present. Other common findings are pleural effusion, arthritis, and ascites. Patients exhibit immunodeficiency secondary to the neoplastic process, in the majority of cases the expression of B-cells positive for EBV is seen, thought to be a consequence of underlying immune dysfunction.
The clinical course is aggressive with a median survival of less than three years. Patients often succumb to infectious complications, which makes delivery of aggressive chemotherapy difficult.
Definitive Diagnostic Methods
Additional X chromosome
Occasional clonal immunoglobulin gene rearrangement and correlation with EBV+ B-gene