Angioimmunoblastic T-cell lymphoma
Name = PAGENAME
ICD10 = ICD10|C|84|4|c|81 (
ICDO = 9705/3
MeshID = D007119
Angioimmunoblastic T-cell lymphoma (AILT) is a mature
T-cell lymphomawith systemic characterized by a polymorphous lymph nodeinfiltrate showing a marked increase in follicular dendritic cells(FDCs) and high endothelial venules(HEVs) and systemic involvement. It is also known as immunoblastic lymphadenopathy (Lukes-Collins Classification) and AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification) [http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] 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 ] The ICD-O codeis 9705/3.
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. AILT comprises 15-20% of peripheral T-cell lymphomas and 1-2% of all non-Hodgkin lymphomas. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9166827&query_hl=8&itool=pubmed_ExternalLink] Anon. "A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project." Blood. 1997 Jun 1;89(11):3909-18. PMID 9166827]
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-cellthat arises "de novo", although some researchers argue that there is a premalignant subtype of this disease. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2642571&query_hl=22&itool=pubmed_ExternalLink] Frizzera G, Kaneko Y, Sakurai M. "Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions." Leukemia. 1989 Jan;3(1):1-5. PMID 2642571] [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10666395&query_hl=26&itool=pubmed_ExternalLink] Smith JL, Hodges E, Quin CT, McCarthy KP, Wright DH. "Frequent T and B cell oligoclones in histologically and immunophenotypically characterized angioimmunoblastic lymphadenopathy." Am J Pathol. 2000 Feb;156(2):661-9. PMID 10666395] The Epstein Barr virus (EBV) is observed in the majority of cases, and the virus has been found in the reactive B-cells that comprise part of the polymorphous infiltrate of this disease [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=1373088&query_hl=15&itool=pubmed_ExternalLink] Weiss LM, Jaffe ES, Liu XF, Chen YY, Shibata D, Medeiros LJ. "Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma." Blood. 1992 Apr 1;79(7):1789-95. PMID 1373088] and in the neoplastic T-cells. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=1327284&query_hl=19&itool=pubmed_ExternalLink] Anagnostopoulos I, Hummel M, Finn T, Tiemann M, Korbjuhn P, Dimmler C, Gatter K, Dallenbach F, Parwaresch MR, Stein H. "Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type." Blood. 1992 Oct 1;80(7):1804-12. PMID 1327284] Immunodeficiency is also seen with this disease, but it is thought to be a sequela to the condition and not a predisposing factor.
Patients with this disease usually present at an advanced stage and show systemic involvement. The
clinical findingstypically include a pruritic skin rash and possibly edema, ascites, pleural effusions, and arthritis. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8664186&query_hl=11&itool=pubmed_ExternalLink] Siegert W, Nerl C, Agthe A, Engelhard M, Brittinger G, Tiemann M, Lennert K, Huhn D. "Angioimmunoblastic lymphadenopathy (AILD)-type T-cell lymphoma: prognostic impact of clinical observations and laboratory findings at presentation. The Kiel Lymphoma Study Group." Ann Oncol. 1995 Sep;6(7):659-64. PMID 8664186] [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8664181&query_hl=13&itool=pubmed_ExternalLink] Jaffe ES. "Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains." Ann Oncol. 1995 Sep;6(7):631-2. PMID 8664181]
The classical laboratory finding is polyclonal
hypergammaglobulinemia, and other immunoglobulinderrangements are also seen, including hemolytic anemiawith cold agglutinins, circulating immune complexes, anti-smooth muscle antibodies, and positive rheumatoid factor.
ites of Involvement
Due to the systemic nature of this disease, neoplastic cells can be found in
lymph nodes, liver, spleen, skin, and bone marrow.
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-cellsare occasionally seen. A classic morphological finding is the aborization and proliferation of high endothelial venules. Hyperplastic germinal centersand Reed-Sternberg cellscan also be seen. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10524524&query_hl=30&itool=pubmed_ExternalLink] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "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. 1999 Oct;23(10):1233-40. PMID 10524524] [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9630171&query_hl=32&itool=pubmed_ExternalLink] Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS. "Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers." Am J Surg Pathol. 1998 Jun;22(6):643-55. PMID 9630171]
T-cell receptorgene rearrangements are detected in 75% of cases [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=2849301&query_hl=34&itool=pubmed_ExternalLink] Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, Kuse R, Mak TW, Lennert K. "Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy." Am J Pathol. 1988 Dec;133(3):549-56. PMID 2849301] , and immunoglobingene rearrangements are seen in 10% of cases, and these cases are believed to be due to expanded EBV-driven B-cellpopulations. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3805286&query_hl=36&itool=pubmed_ExternalLink] Lipford EH, Smith HR, Pittaluga S, Jaffe ES, Steinberg AD, Cossman J. "Clonality of angioimmunoblastic lymphadenopathy and implications for its evolution to malignant lymphoma." J Clin Invest. 1987 Feb;79(2):637-42. PMID 3805286] Similarly, EBV-related sequences can be detected most cases, usually in B-cellsbut occasionally in T-cells.. Trisomy3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases. [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3261178&query_hl=40&itool=pubmed_ExternalLink] Kaneko Y, Maseki N, Sakurai M, Takayama S, Nanba K, Kikuchi M, Frizzera G. "Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features." Blood. 1988 Aug;72(2):413-21. PMID 3261178] [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7919378&query_hl=42&itool=pubmed_ExternalLink] Schlegelberger B, Zhang Y, Weber-Matthiesen K, Grote W. "Detection of aberrant clones in nearly all cases of angioimmunoblastic lymphadenopathy with dysproteinemia-type T-cell lymphoma by combined interphase and metaphase cytogenetics." Blood. 1994 Oct 15;84(8):2640-8. PMID 7919378]
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