Angioimmunoblastic T-cell lymphoma
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| Angioimmunoblastic T-cell lymphoma Classification and external resources | |
| ICD-10 | C84.4 (ILDS C84.460) |
|---|---|
| ICD-O: | 9705/3 |
| MeSH | D007119 |
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Overview
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]
Epidemiology
The 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.[1]
Clinical Features
Etiology
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[1], although some researchers argue that there is a premalignant subtype of this disease.[1][1] 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[1] and in the neoplastic T-cells.[1] 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 Presentation
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.[1][1]
Laboratory Findings
The 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.[1][1]
Sites of Involvement
Due to the systemic nature of this disease, neoplastic cells can be found in lymph nodes, liver, spleen, skin, and bone marrow.
Morphology
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 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.[1][1]
Molecular Findings
Immunophenotype
AILT 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 Findings
Clonal T-cell receptor gene rearrangements are detected in 75% of cases[1], 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.[1] Similarly, EBV-related sequences can be detected most cases, usually in B-cells but occasionally in T-cells.[1][1]. Trisomy 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.[1][1]
References
WikiDoc Research Resources for Angioimmunoblastic T-cell lymphoma | |
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| Articles on Angioimmunoblastic T-cell lymphoma | Most recent articles on Angioimmunoblastic T-cell lymphoma • Most cited articles on Angioimmunoblastic T-cell lymphoma • Review articles on Angioimmunoblastic T-cell lymphoma • Articles on Angioimmunoblastic T-cell lymphoma in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Angioimmunoblastic T-cell lymphoma | Powerpoint slides on Angioimmunoblastic T-cell lymphoma • Images of Angioimmunoblastic T-cell lymphoma • Photos of Angioimmunoblastic T-cell lymphoma • Podcasts & MP3s on Angioimmunoblastic T-cell lymphoma • Videos on Angioimmunoblastic T-cell lymphoma |
| Evidence Based Medicine Regarding Angioimmunoblastic T-cell lymphoma | Cochrane Collaboration on Angioimmunoblastic T-cell lymphoma • Bandolier on Angioimmunoblastic T-cell lymphoma • TRIP on Angioimmunoblastic T-cell lymphoma |
| Cost Effectiveness of Angioimmunoblastic T-cell lymphoma | Cost Effectiveness of Angioimmunoblastic T-cell lymphoma |
| Clinical Trials Involving Angioimmunoblastic T-cell lymphoma | Ongoing Trials on Angioimmunoblastic T-cell lymphoma at Clinical Trials.gov • Trial results on Angioimmunoblastic T-cell lymphoma • Clinical Trials on Angioimmunoblastic T-cell lymphoma at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Angioimmunoblastic T-cell lymphoma | US National Guidelines Clearinghouse on Angioimmunoblastic T-cell lymphoma • NICE Guidance on Angioimmunoblastic T-cell lymphoma • NHS PRODIGY Guidance • FDA on Angioimmunoblastic T-cell lymphoma • CDC on Angioimmunoblastic T-cell lymphoma |
| Textbook Information on Angioimmunoblastic T-cell lymphoma | Books and Textbook Information on Angioimmunoblastic T-cell lymphoma |
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| Genetics, Pharmacogenomics, and Proteinomics of Angioimmunoblastic T-cell lymphoma | Genetics of Angioimmunoblastic T-cell lymphoma • Pharmacogenomics of Angioimmunoblastic T-cell lymphoma • Proteomics of Angioimmunoblastic T-cell lymphoma |
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| Commentary on Angioimmunoblastic T-cell lymphoma | Blogs on Angioimmunoblastic T-cell lymphoma |
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| Healthcare Provider Resources on Angioimmunoblastic T-cell lymphoma | Symptoms of Angioimmunoblastic T-cell lymphoma • Causes & Risk Factors for Angioimmunoblastic T-cell lymphoma • Diagnostic studies for Angioimmunoblastic T-cell lymphoma • Treatment of Angioimmunoblastic T-cell lymphoma |
| Continuing Medical Education (CME) Programs on Angioimmunoblastic T-cell lymphoma | CME Programs on Angioimmunoblastic T-cell lymphoma |
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| Informatics Resources on Angioimmunoblastic T-cell lymphoma | List of terms related to Angioimmunoblastic T-cell lymphoma |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
