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Angioimmunoblastic T-cell lymphoma
Angioimmunoblastic T-cell lymphoma (AILT) is a mature T-cell lymphoma with systemic 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] It is also known as immunoblastic lymphadenopathy (Lukes-Collins Classification) and AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification)[1] The ICD-O code is 9705/3.[1] Additional recommended knowledge
EpidemiologyThe typical patient with angioimmunoblastic T-cell lymphoma (AILT) is either middle-aged or elderly, and no gender preference for this disease has been observed.[1] AILT comprises 15-20% of peripheral T-cell lymphomas and 1-2% of all non-Hodgkin lymphomas.[2] Clinical FeaturesEtiologyThis 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[1], although some researchers argue that there is a premalignant subtype of this disease.[3][4] The Epstein Barr virus (EBV) is observed in the majority of cases[1], and the virus has been found in the reactive B-cells that comprise part of the polymorphous infiltrate of this disease[5] and in the neoplastic T-cells.[6] Immunodeficiency is also seen with this disease, but it is thought to be a sequela to the condition and not a predisposing factor.[1] Clinical PresentationPatients 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.[7][8] Laboratory FindingsThe classical laboratory finding is polyclonal hypergammaglobulinemia, and other immunoglobulin derrangements are also seen, including hemolytic anemia with cold agglutinins, circulating immune complexes, anti-smooth muscle antibodies, and positive rheumatoid factor.[7][1] Sites of InvolvementDue to the systemic nature of this disease, neoplastic cells can be found in lymph nodes, liver, spleen, skin, and bone marrow. MorphologyLymph nodeThe 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 lymphocytes, eosinophils, histiocytes, plasma cells, and follicular dendritic cells. In addition, blast-like B-cells are occasionally seen. A classic morphological finding is the aborization and proliferation of high endothelial venules.[1] Hyperplastic germinal centers and Reed-Sternberg cells can also be seen.[9][10] Molecular FindingsImmunophenotypeAILT typically has the phenotype of a mixture of CD4+ and CD8+ T-cells, with a CD4:CD8 ratio greater than unity. Polyclonal plasma cells and CD21+ follicular dendritic cells are also seen.[1] Genetic FindingsClonal T-cell receptor gene rearrangements are detected in 75% of cases[11], and immunoglobin gene rearrangements are seen in 10% of cases, and these cases are believed to be due to expanded EBV-driven B-cell populations.[12] Similarly, EBV-related sequences can be detected most cases, usually in B-cells but occasionally in T-cells.[5][6]. Trisomy 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.[13][14] References
Categories: Hematology | Types of cancer | Blood disorders |
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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Angioimmunoblastic_T-cell_lymphoma". A list of authors is available in Wikipedia. |
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