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</ref> Similarly, EBV-related sequences can be detected most cases, usually in [[B-cells]] but occasionally in [[T-cells]].<ref name=wei1/><ref name=ana1/>. [[Trisomy]] 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.<ref name=kane1>{{cite journal |author=Kaneko Y, Maseki N, Sakurai M, ''et al.'' |title=Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features |journal=Blood |volume=72 |issue=2 |pages=413–21 |year=1988 |month=August |pmid=3261178 }}</ref>
</ref> Similarly, EBV-related sequences can be detected most cases, usually in [[B-cells]] but occasionally in [[T-cells]].<ref name=wei1/><ref name=ana1/>. [[Trisomy]] 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.<ref name=kane1>{{cite journal |author=Kaneko Y, Maseki N, Sakurai M, ''et al.'' |title=Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features |journal=Blood |volume=72 |issue=2 |pages=413–21 |year=1988 |month=August |pmid=3261178 }}</ref>
<ref name=sch1>{{cite journal |author=Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W |title=Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics |journal=Blood |volume=84 |issue=8 |pages=2640–8 |year=1994 |month=October |pmid=7919378 }}</ref>
<ref name=sch1>{{cite journal |author=Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W |title=Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics |journal=Blood |volume=84 |issue=8 |pages=2640–8 |year=1994 |month=October |pmid=7919378 }}</ref>

==Treatment==

Currently AITL, being a subtype of PTCL, is treated similarly to B-cell lymphomas. However, in recent years, scientists have developed techniques to better recognize the different types of lymphomas, such as PTCL. It is now understood that PTCL behaves differently from B-cell lymphomas and therapies are being developed that specifically target these types of lymphoma. Currently, however, there are no [[FDA]]-approved therapies specifically for PTCL. [[Anthracycline]]-containing [[chemotherapy regimens]] are commonly offered as the initial therapy. Some patients may receive a [[stem cell transplant]].<ref>Reimer P, et al. ''J Clin Oncol.'' 2009;27:106-113</ref><ref>Mercadal S, et al. ''Ann Oncol.'' 2008;19:958-963</ref><ref>Rodriguez J, et al. ''Eur J Haematol.'' 2007;79:32-38</ref><ref>Corradini P, et al. ''Leukemia.'' 2006;20:1533-1538</ref><ref>d’Amore F, et al. ''Blood.'' 2006;108:A401</ref><ref>Gisselbrecht C, et al. ''J Clin Oncol.'' 2002;20:2472-2479</ref><ref>Deconinck E, et al. ''Br J Haematol.'' 2000;109:736-742</ref><ref>Haioun C, et al. ''J Clin Oncol.'' 2000;18:3025-3030</ref> Novel approaches to the treatment of PTCL in the relapsed or refractory setting are under investigation.

[[Pralatrexate]] is one compound currently under investigations for the treatment of PTCL. For information please consult the US clinical trials database ([http://www.clinicaltrials.gov http://www.clinicaltrials.gov]).


== See also ==
== See also ==

Revision as of 12:42, 17 July 2009

Angioimmunoblastic T-cell lymphoma
SpecialtyOncology Edit this on Wikidata

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]

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]

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.[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]

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]

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]

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.[10][11]

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[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]

See also

References

  1. ^ 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
  2. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. {{cite book}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
  3. ^ "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. 1997. PMID 9166827. {{cite journal}}: Unknown parameter |month= ignored (help)
  4. ^ Frizzera G, Kaneko Y, Sakurai M (1989). "Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions". Leukemia. 3 (1): 1–5. PMID 2642571. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  5. ^ Smith JL, Hodges E, Quin CT, McCarthy KP, Wright DH (2000). "Frequent T and B cell oligoclones in histologically and immunophenotypically characterized angioimmunoblastic lymphadenopathy". Am. J. Pathol. 156 (2): 661–9. PMC 1850038. PMID 10666395. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  6. ^ a b Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ (1992). "Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma". Blood. 79 (7): 1789–95. PMID 1373088. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  7. ^ a b Anagnostopoulos I, Hummel M, Finn T; et al. (1992). "Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type". Blood. 80 (7): 1804–12. PMID 1327284. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  8. ^ a b Siegert W, Nerl C, Agthe A; et al. (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. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  9. ^ Jaffe ES (1995). "Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains". Ann. Oncol. 6 (7): 631–2. PMID 8664181. {{cite journal}}: Unknown parameter |month= ignored (help)
  10. ^ Quintanilla-Martinez L, Fend F, Moguel LR; et al. (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. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  11. ^ Ree HJ, Kadin ME, Kikuchi M; et al. (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. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  12. ^ Feller AC, Griesser H, Schilling CV; et al. (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. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  13. ^ Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J (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. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  14. ^ Kaneko Y, Maseki N, Sakurai M; et al. (1988). "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features". Blood. 72 (2): 413–21. PMID 3261178. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  15. ^ Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W (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. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
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