Angioimmunoblastic T-cell lymphoma explained

Angioimmunoblastic T-cell lymphoma
Synonyms:immunoblastic lymphadenopathy (Lukes-Collins Classification), AILD-type (lymphogranulomatosis X) T-cell lymphoma (Kiel Classification)[1]
Field:Hematology and oncology

Angioimmunoblastic T-cell lymphoma (AITL, sometimes misspelled AILT, formerly known as "angioimmunoblastic lymphadenopathy with dysproteinemia"[2]) is a mature T-cell lymphoma of blood or lymph vessel immunoblasts 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]

Signs and symptoms

Patients with AITL 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.[3] [4]

Causes

AITL was originally thought to be a premalignant condition, termed angioimmunoblastic lymphadenopathy, and this atypical reactive lymphadenopathy carried a risk for transformation into a lymphoma. It is postulated that the originating cell for AITL is a mature (post-thymic) CD4+ T-cell that arises de novo,[1] or that the disease has a premalignant subtype.[5] [6] The Epstein–Barr virus (EBV) is observed in the majority of cases,[1] being identified in the reactive (i.e. non-malignant) B-cells that comprise part of the polymorphous infiltrate of AITL.[7] These EBV+ B cells have numerous non-malignant crippling mutations, often proliferate excessively, and in some cases may transform into EBV+ B cell lymphomas. The other cell types in these infiltrates, including the malignant TFH cells, are EBV negative. While the World Health Organization (2016) has classified these EBV-associated cases as one of the Epstein-Barr virus-associated lymphoproliferative diseases (see EBV+ angioimmunoblastic T cell lymphoma, the role of the virus in the development and progression of EBV+ angioimmunoblastic T cell lymphoma is unclear.[8] Immunodeficiency is also seen with AITL, but it is a sequela and not a predisposing factor.[1]

Diagnosis

Laboratory findings

The classical laboratory finding is polyclonal hypergammaglobulinemia, and other immunoglobulin derangements are also seen, including hemolytic anemia with cold agglutinins, circulating immune complexes, anti-smooth muscle antibodies, and positive rheumatoid factor.[1] [3]

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-like cells can also be seen.[9] [10]

Immunophenotype

AITL 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]

Molecular findings

Clonal T-cell receptor gene rearrangements are detected in 75% of cases,[11] 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.[12] Similarly, EBV-related sequences can be detected in most cases, usually in B-cells but occasionally in T-cells.[7] [13] Trisomy 3, trisomy 5, and +X are the most frequent chromosomal abnormalities found in AITL cases.[14] [15]

Epidemiology

The typical patient with angioimmunoblastic T-cell lymphoma (AITL) is either middle-aged or elderly, and no gender preference for this disease has been observed.[1] AITL comprises 15–20% of peripheral T-cell lymphomas and 1–2% of all non-Hodgkin lymphomas.[16]

See also

Notes and References

  1. Book: Swerdlow . S.H. . Campo . E. . Harris . N.L. . Jaffe . E.S. . Pileri . S.A. . Stein . H. . Thiele . J. . Vardiman . J.W . 11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma . WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues . 4th . IARC WHO Classification of Tumours . 2 . IARC . 2008 . 978-9283224310 . http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002&content=1.
  2. Book: James . William D. . Berger . Timothy G. . Andrews' Diseases of the Skin: Clinical Dermatology . Saunders Elsevier . 2006 . 0-7216-2921-0 . etal.
  3. Siegert W, Nerl C, Agthe A . 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 . September 1995. 8664186 . etal. 10.1093/oxfordjournals.annonc.a059281 . free .
  4. Jaffe ES . Angioimmunoblastic T-cell lymphoma: new insights, but the clinical challenge remains . Ann. Oncol. . 6 . 7 . 631–2 . September 1995. 8664181 . 10.1093/oxfordjournals.annonc.a059273 . free .
  5. Frizzera G, Kaneko Y, Sakurai M . Angioimmunoblastic lymphadenopathy and related disorders: a retrospective look in search of definitions . Leukemia . 3 . 1 . 1–5 . January 1989. 2642571 .
  6. 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. . 156 . 2 . 661–9 . February 2000. 10666395 . 1850038 . 10.1016/S0002-9440(10)64770-0 .
  7. 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 . 79 . 7 . 1789–95 . April 1992. 10.1182/blood.V79.7.1789.1789 . 1373088 . free .
  8. Rezk SA, Zhao X, Weiss LM . Epstein-Barr virus (EBV)-associated lymphoid proliferations, a 2018 update . Human Pathology . 79 . 18–41 . September 2018 . 29885408 . 10.1016/j.humpath.2018.05.020 . 47010934 .
  9. Quintanilla-Martinez L, Fend F, Moguel LR . 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 . October 1999. 10524524 . 10.1097/00000478-199910000-00008 . etal.
  10. Ree HJ, Kadin ME, Kikuchi M . Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers . Am. J. Surg. Pathol. . 22 . 6 . 643–55 . June 1998. 9630171 . 10.1097/00000478-199806000-00001 . etal.
  11. Feller AC, Griesser H, Schilling CV . Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy . Am. J. Pathol. . 133 . 3 . 549–56 . December 1988. 2849301 . 1880823 . etal.
  12. 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. . 79 . 2 . 637–42 . February 1987. 3805286 . 424152 . 10.1172/JCI112860 .
  13. Anagnostopoulos I, Hummel M, Finn T . Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type . Blood . 80 . 7 . 1804–12 . October 1992. 10.1182/blood.V80.7.1804.1804 . 1327284 . etal . free .
  14. Kaneko Y, Maseki N, Sakurai M . Characteristic karyotypic pattern in T-cell lymphoproliferative disorders with reactive "angioimmunoblastic lymphadenopathy with dysproteinemia-type" features . Blood . 72 . 2 . 413–21 . August 1988. 10.1182/blood.V72.2.413.413 . 3261178 . etal . free .
  15. 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 . 84 . 8 . 2640–8 . October 1994. 10.1182/blood.V84.8.2640.2640 . 7919378 . free .
  16. 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. 9166827 . 10.1182/blood.V89.11.3909. free .

This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Angioimmunoblastic T-cell lymphoma".

Except where otherwise indicated, Everything.Explained.Today is © Copyright 2009-2024, A B Cryer, All Rights Reserved. Cookie policy.