Angioimmunoblastic T-cell lymphoma: Difference between revisions
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Revision as of 15:36, 31 July 2012
| Angioimmunoblastic T-cell lymphoma | |
| ICD-10 | C84.4 (ILDS C84.460) |
|---|---|
| ICD-O: | 9705/3 |
| MeSH | D007119 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [15]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [16]
Synonyms and keywords: AILD-type (lymphogranulomatosis X) T-cell lymphoma, AILT, immunoblastic lymphadenopathy
Overview
Angioimmunoblastic T-cell lymphoma (AILT) is a mature T-cell lymphoma 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].
Pathophysiology
Genetics
Clonal T-cell receptor gene rearrangements are detected in 75% of cases[2], 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.[3] Similarly, EBV-related sequences can be detected most cases, usually in B-cells but occasionally in T-cells.[4][5]. Trisomy 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in cases.[6][7]
Gross Pathology
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 reactivelymphocytes, eosinophils, histiocytes, plasma cells, and follicular dendritic cells. In addition, blast-likeB-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.[8][9]
Microscopic Pathology
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] Due to the systemic nature of this disease, neoplastic cells can be found in lymph nodes, liver, spleen, skin, and bone marrow.
Causes
AILT 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.[10][11] 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[4] and in the neoplastic T-cells.[5] Immunodeficiency is also seen with this disease, but it is thought to be a sequela to the condition and not a predisposing factor.[1]
Epidemiology and Demographics
- AILT comprises 15-20% of peripheral T-cell lymphomas and 1-2% of all non-Hodgkin lymphomas.[12]
- The typical patient with angioimmunoblastic T-cell lymphoma (AILT) is either middle-aged or elderly
- No gender preference for this disease has been observed.[1]
Diagnosis
Symptoms
Patients with this disease usually present at an advanced stage and show systemic involvement. The clinical findings typically include
Laboratory Findings
The classical laboratory finding is polyclonal hypergammaglobulinemia
Other immunoglobulin derrangements are also seen, including
- Hemolytic anemia with cold agglutinins
- Circulating immune complexes
- Anti-smooth muscle antibodies
- Positive rheumatoid factor.[13][1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 [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] 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
- ↑ [3] 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
- ↑ 4.0 4.1 [4] 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
- ↑ 5.0 5.1 [5] 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
- ↑ [6] 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
- ↑ [7] 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
- ↑ [8] 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
- ↑ [9] 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
- ↑ [10] 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
- ↑ [11] 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
- ↑ [12] 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
- ↑ 13.0 13.1 [13] 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
- ↑ [14] Jaffe ES. "Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains." Ann Oncol. 1995 Sep;6(7):631-2. PMID: 8664181