Clinical manifestations, pathologic features, and diagnosis of angioimmunoblastic T cell lymphoma
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- Jon C Aster, MD
Jon C Aster, MD
- Professor of Pathology
- Harvard Medical School
The peripheral T cell lymphomas (PTCL) are a heterogeneous group of generally aggressive neoplasms that constitute less than 15 percent of all non-Hodgkin lymphomas (NHLs) in adults [1,2]. (See "Classification of the hematopoietic neoplasms".)
Among these, in decreasing frequency of occurrence, are:
●Peripheral T cell lymphoma, not otherwise specified
●Anaplastic large cell lymphoma, primary systemic type
●Angioimmunoblastic T cell lymphoma
- World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC Press, Lyon 2008.
- Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127:2375.
- Grogg KL, Attygalle AD, Macon WR, et al. Angioimmunoblastic T-cell lymphoma: a neoplasm of germinal-center T-helper cells? Blood 2005; 106:1501.
- Krenacs L, Schaerli P, Kis G, Bagdi E. Phenotype of neoplastic cells in angioimmunoblastic T-cell lymphoma is consistent with activated follicular B helper T cells. Blood 2006; 108:1110.
- 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.
- Rudiger T, Weisenburger DD, Coiffier B, et al. Angioimmunoblastic T-cell Lymphoma: A report from the international peripheral T-cell lymphoma project. Ann Oncol 2008; 19(suppl 4):iv119.
- Lennert K, Feller A. Histopathology of non-Hodgkin's lymphomas, Springer-Verlag, New York 1992.
- Morton LM, Wang SS, Devesa SS, et al. Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001. Blood 2006; 107:265.
- Adams SV, Newcomb PA, Shustov AR. Racial Patterns of Peripheral T-Cell Lymphoma Incidence and Survival in the United States. J Clin Oncol 2016; 34:963.
- Ohsaka A, Saito K, Sakai T, et al. Clinicopathologic and therapeutic aspects of angioimmunoblastic lymphadenopathy-related lesions. Cancer 1992; 69:1259.
- Pautier P, Devidas A, Delmer A, et al. Angioimmunoblastic-like T-cell non Hodgkin's lymphoma: outcome after chemotherapy in 33 patients and review of the literature. Leuk Lymphoma 1999; 32:545.
- Siegert W, Nerl C, Agthe A, et al. 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; 6:659.
- Federico M, Rudiger T, Bellei M, et al. Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the international peripheral T-cell lymphoma project. J Clin Oncol 2013; 31:240.
- Dogan A, Morice WG. Bone marrow histopathology in peripheral T-cell lymphomas. Br J Haematol 2004; 127:140.
- Seehafer JR, Goldberg NC, Dicken CH, Su WP. Cutaneous manifestations of angioimmunoblastic lymphadenopathy. Arch Dermatol 1980; 116:41.
- Park BB, Ryoo BY, Lee JH, et al. Clinical features and treatment outcomes of angioimmunoblastic T-cell lymphoma. Leuk Lymphoma 2007; 48:716.
- Dogan A, Attygalle AD, Kyriakou C. Angioimmunoblastic T-cell lymphoma. Br J Haematol 2003; 121:681.
- Sallah S, Gagnon GA. Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment. Acta Haematol 1998; 99:57.
- Grogg KL, Morice WG, Macon WR. Spectrum of bone marrow findings in patients with angioimmunoblastic T-cell lymphoma. Br J Haematol 2007; 137:416.
- Lee SS, Rüdiger T, Odenwald T, et al. Angioimmunoblastic T cell lymphoma is derived from mature T-helper cells with varying expression and loss of detectable CD4. Int J Cancer 2003; 103:12.
- Feller AC, Griesser H, Schilling CV, et al. Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy. Am J Pathol 1988; 133:549.
- Attygalle A, Al-Jehani R, Diss TC, et al. Neoplastic T cells in angioimmunoblastic T-cell lymphoma express CD10. Blood 2002; 99:627.
- Mourad N, Mounier N, Brière J, et al. Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials. Blood 2008; 111:4463.
- Weiss LM, Strickler JG, Dorfman RF, et al. Clonal T-cell populations in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma. Am J Pathol 1986; 122:392.
- Anagnostopoulos I, Hummel M, Finn T, et al. Heterogeneous Epstein-Barr virus infection patterns in peripheral T-cell lymphoma of angioimmunoblastic lymphadenopathy type. Blood 1992; 80:1804.
- Weiss LM, Jaffe ES, Liu XF, et al. Detection and localization of Epstein-Barr viral genomes in angioimmunoblastic lymphadenopathy and angioimmunoblastic lymphadenopathy-like lymphoma. Blood 1992; 79:1789.
- Zhou Y, Attygalle AD, Chuang SS, et al. Angioimmunoblastic T-cell lymphoma: histological progression associates with EBV and HHV6B viral load. Br J Haematol 2007; 138:44.
- Schlegelberger B, Feller A, Gödde E, et al. Stepwise development of chromosomal abnormalities in angioimmunoblastic lymphadenopathy. Cancer Genet Cytogenet 1990; 50:15.
- Wang C, McKeithan TW, Gong Q, et al. IDH2R172 mutations define a unique subgroup of patients with angioimmunoblastic T-cell lymphoma. Blood 2015; 126:1741.
- Lemonnier F, Couronné L, Parrens M, et al. Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters. Blood 2012; 120:1466.
- Cairns RA, Iqbal J, Lemonnier F, et al. IDH2 mutations are frequent in angioimmunoblastic T-cell lymphoma. Blood 2012; 119:1901.
- Odejide O, Weigert O, Lane AA, et al. A targeted mutational landscape of angioimmunoblastic T-cell lymphoma. Blood 2014; 123:1293.
- Palomero T, Couronné L, Khiabanian H, et al. Recurrent mutations in epigenetic regulators, RHOA and FYN kinase in peripheral T cell lymphomas. Nat Genet 2014; 46:166.
- Sakata-Yanagimoto M, Enami T, Yoshida K, et al. Somatic RHOA mutation in angioimmunoblastic T cell lymphoma. Nat Genet 2014; 46:171.
- Yoo HY, Sung MK, Lee SH, et al. A recurrent inactivating mutation in RHOA GTPase in angioimmunoblastic T cell lymphoma. Nat Genet 2014; 46:371.
- International Non-Hodgkin's Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin's lymphoma. N Engl J Med 1993; 329:987.
- Gallamini A, Stelitano C, Calvi R, et al. Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study. Blood 2004; 103:2474.
- Cuadros M, Dave SS, Jaffe ES, et al. Identification of a proliferation signature related to survival in nodal peripheral T-cell lymphomas. J Clin Oncol 2007; 25:3321.
- Tokunaga T, Shimada K, Yamamoto K, et al. Retrospective analysis of prognostic factors for angioimmunoblastic T-cell lymphoma: a multicenter cooperative study in Japan. Blood 2012; 119:2837.
- Willenbrock K, Bräuninger A, Hansmann ML. Frequent occurrence of B-cell lymphomas in angioimmunoblastic T-cell lymphoma and proliferation of Epstein-Barr virus-infected cells in early cases. Br J Haematol 2007; 138:733.
- CLINICAL FEATURES
- PATHOLOGIC FEATURES
- - Growth pattern
- - Cell morphology
- - Bone marrow involvement
- Genetic features
- DIFFERENTIAL DIAGNOSIS
- Other peripheral T cell lymphomas
- Hodgkin lymphoma
- EBV-positive diffuse large B cell lymphoma, NOS