Angioimmunoblastic T-cell lymphoma
| Angioimmunoblastic T-cell lymphoma | |
|---|---|
| Classification and external resources | |
| ICD-10 | C84.4 (ILDS C84.460) |
| ICD-O: | 9705/3[1] |
| MeSH | D007119 |
Angioimmunoblastic T-cell lymphoma (AILT) (also known as "Angioimmunoblastic lymphadenopathy with dysproteinemia"[2]:747) 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]
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[edit] 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.[3]
[edit] Clinical features
[edit] 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.[4][5] 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[6] and in the neoplastic T-cells.[7] Immunodeficiency is also seen with this disease, but it is a sequela to the condition and not a predisposing factor.[1]
[edit] 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.[8][9]
[edit] 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.[8][1]
[edit] 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.
[edit] Morphology
[edit] 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.[10][11]
[edit] Molecular findings
[edit] 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]
[edit] Genetic findings
Clonal T-cell receptor gene rearrangements are detected in 75% of cases[12], 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.[13] Similarly, EBV-related sequences can be detected most cases, usually in B-cells but occasionally in T-cells.[6][7]. Trisomy 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.[14] [15]
[edit] See also
[edit] References
- ^ a b c d e f g h i j [1] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001
- ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ^ "A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project". Blood 89 (11): 3909–18. June 1997. PMID 9166827.
- ^ Frizzera G, Kaneko Y, Sakurai M (January 1989). "Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions". Leukemia 3 (1): 1–5. PMID 2642571.
- ^ Smith JL, Hodges E, Quin CT, McCarthy KP, Wright DH (February 2000). "Frequent T and B Cell Oligoclones in Histologically and Immunophenotypically Characterized Angioimmunoblastic Lymphadenopathy". Am. J. Pathol. 156 (2): 661–9. doi:10.1016/S0002-9440(10)64770-0. PMC 1850038. PMID 10666395. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1850038.
- ^ a b Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ (April 1992). "Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma". Blood 79 (7): 1789–95. PMID 1373088.
- ^ a b Anagnostopoulos I, Hummel M, Finn T, et al. (October 1992). "Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type". Blood 80 (7): 1804–12. PMID 1327284.
- ^ a b Siegert W, Nerl C, Agthe A, et al. (September 1995). "Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation. The Kiel Lymphoma Study Group". Ann. Oncol. 6 (7): 659–64. PMID 8664186.
- ^ Jaffe ES (September 1995). "Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains". Ann. Oncol. 6 (7): 631–2. PMID 8664181.
- ^ Quintanilla-Martinez L, Fend F, Moguel LR, et al. (October 1999). "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection". Am. J. Surg. Pathol. 23 (10): 1233–40. doi:10.1097/00000478-199910000-00008. PMID 10524524.
- ^ Ree HJ, Kadin ME, Kikuchi M, et al. (June 1998). "Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers". Am. J. Surg. Pathol. 22 (6): 643–55. doi:10.1097/00000478-199806000-00001. PMID 9630171.
- ^ Feller AC, Griesser H, Schilling CV, et al. (December 1988). "Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy". Am. J. Pathol. 133 (3): 549–56. PMC 1880823. PMID 2849301. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1880823.
- ^ Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J (February 1987). "Clonality of angioimmunoblastic lymphadenopathy and implications for its evolution to malignant lymphoma". J. Clin. Invest. 79 (2): 637–42. doi:10.1172/JCI112860. PMC 424152. PMID 3805286. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=424152.
- ^ Kaneko Y, Maseki N, Sakurai M, et al. (August 1988). "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features". Blood 72 (2): 413–21. PMID 3261178.
- ^ Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W (October 1994). "Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics". Blood 84 (8): 2640–8. PMID 7919378.
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