Angioimmunoblastic T-cell lymphoma (AITL)

Angioimmunoblastic T-cell lymphoma (AITL)  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.

Immunophenotyping:

Positive  for Pan T cell antigens( CD2, CD3, CD5 and CD7 with frequent aberrant loss of one or more of these antigens),T helper cell marker( CD4), Follicular T helper cell markers (CD10, BCL6, CXCL13 , PD-1 (CD279), ICOS),Follicular dendritic cell networks (CD21, CD23, CD35, CNA.42 or clusterin), Scattered B cells: EBV+ in Lymph nodes

Uncommon expression of CD10 (25%), Lack of CXCL13 expression, PD-1 in 85%, BCL6 / CD3 dual staining in Bone marrow:

Negative for CD8, CD56, pan B cell markers, immunoglobulins, cytotoxic antigens(TCRαβ, TCR γδ), CD1a, CD99, TdT,

 

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Figure shows The neoplastic cells have the same light scatter distribution as normal T cells in FS/SS PLOT.  Immunophenotyping shows CD7+(dim), CD8-,TCRαβ-, TCR γδ+  CD3 dim, CD16+, CD56-, CD2+, CD20-, CD22-, CD5+, CD38dm, CD10+, CD4+.

 

References

  • Dogan A, Gaulard P, Jaffe ES, Ralfkiaer E. Angioimmunoblastic T-cell lymphoma. In: Swerdlow SH, Campo E, Harris NL, et al., editors. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2008. p. 309–311. [Google Scholar]

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  • de Leval L, Rickman DS Thielen C, et al. The gene expression profile of nodal peripheral T-cell lymphoma demonstrates a molecular link between angioimmunoblastic T-cell lymphoma (AITL) and follicular helper T (TFH) cells. Blood. 2007;109:4952–4963. [PubMed]

  • Piccaluga PP, Fuligni F De Leo A. Molecular profiling improves classification and prognostication of nodal peripheral T-cell lymphomas: results of a phase III diagnostic accuracy study. J Clin Oncol. 2013;31:3019–3025.[PubMed]

  • Lee PS, Lin CN, Chuang SS. Immunophenotyping of angioimmunoblastic T-cell lymphomas by multiparameter flow cytometry. Pathol Res Pract. 2003;199:539–545. [PubMed]

  • Baseggio L, Berger F, Morel D, et al. Identification of circulating CD10 positive T cells in angioimmunoblastic T-cell lymphoma. Leukemia. 2006;20:296–303.

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  • Stacchini A, Demurtas A, Aliberti S, et al. The usefulness of flow cytometric CD10 detection in the differential diagnosis of peripheral T-cell lymphomas. Am J Clin Pathol. 2007;128:854–864. [PubMed]

  • Zhang D, Saunders CJ, Zhao W, et al. The clonality of CD3+ CD10+ T cells in angioimmunoblastic T cell lymphoma, B cell lymphoma, and reactive lymphoid hyperplasia. Am J Hematol. 2009;84:606–608. [PubMed]

  • Khokhar FA, Payne WD, Talwalkar SS, et al. Angioimmunoblastic T-cell lymphoma in bone marrow: a morphologic and immunophenotypic study. Hum Pathol. 2010;41:79–87.[PubMed]

  • Baseggio L, Traverse-Glehen A, Berger F, et al. CD10 and ICOS expression by multiparametric flow cytometry in angioimmunoblastic T-cell lymphoma. Mod Pathol. 2011;24:993–1003. [PubMed]

  • Xerri L, Chetaille B, Serriari N, et al. Programmed death 1 is a marker of angioimmunoblastic T-cell lymphoma and B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia. Hum Pathol. 2008;39:1050–1058. [PubMed]

  • Serke S, van Lessen A, Hummel M, et al. Circulating CD4+ T lymphocytes with intracellular but no surface CD3 antigen in five of seven patients consecutively diagnosed with angioimmunoblastic T-cell lymphoma. Cytometry. 2000;42:180–187. [PubMed]

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