Abstract
There are several variant forms of T-cell non-Hodgkin’s lymphomas that can involve the hepatobiliary tract. A distinct and highly aggressive variety of T-cell NHL is the lymphoepithelioid cell variant of peripheral T-cell lymphoma, also termed Lennert’s lymphoma (LECL). LECL is characterized by a proliferation of CD4-positive T cells associated with high content of epithelioid macrophages/histiocytes. LECL exhibits a high incidence of generalized lymphadenopathy but rare extranodal disease. Liver involvement may occur, with variable degrees of hepatomegaly. A variant of peripheral T-cell NHL with a polymorphous T-cell lymphoproliferation and association with EBV infection and systemic disease is angioimmunoblastic T-cell lymphoma. This neoplasm accounts for 15–20 % of T-cell NHL. Hepatosplenomegaly is a frequent feature, and the liver shows infiltration by polymorphous lymphoid cells of the T-cell class associated with angiogenesis.
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References
Abruzzo LV, Schmidt K, Weiss LM, Jaffe ES, Medeiros LJ, Sander CA, Raffeld M (1993) B-cell lymphoma after angioimmunoblastic lymphadenopathy: a case with oligoclonal gene rearrangements associated with Epstein-Barr virus. Blood 82:241–246
Admirand JH, Rassidakis GZ, Abruzzo LV, Valbuena JR, Jones D, Jeffrey Medeiros L (2004) Immunohistochemical detection of ZAP-70 in 341 cases of non-Hodgkin and Hodgkin lymphoma. Mod Pathol 17:954–961
Attygalle A, Al Jehani R, Diss TC, Munson P, Liu H, Du MQ, Isaacson PG, Dogan A (2002) Neoplastic T cells in angioimmunoblastic T-cell lymphoma express CD10. Blood 99:627–633
Attygalle AD, Diss TC, Munson P, Isaacson PG, Du MQ, Dogan A (2004) CD10 expression in extranodal dissemination of angioimmunoblastic T-cell lymphoma. Am J Surg Pathol 28:54–61
Attygalle AD, Kyriakou C, Dupuis J, Grogg KL, Diss TC, Wotherspoon AC, Chuang SS et al (2007a) Histologic evolution of angioimmunoblastic T-cell lymphoma in consecutive biopsies: clinical correlation and insights into natural history and disease progression. Am J Surg Pathol 31:1077–1088
Attygalle AD, Chuang SS, Diss TC, Du MQ, Isaacson PG, Dogan A (2007b) Distinguishing angioimmunoblastic T-cell lymphoma from peripheral T-cell lymphoma, unspecified, using morphology, immunophenotype and molecular genetics. Histopathology 50:498–508
Battegay M, Berger C, Rochlitz C, Hurwitz N, Hirsch HH, de Geyter C, Haque T, Nadal D (2004) Epstein-Barr virus load correlating with clinical manifestation and treatment response in a patient with angioimmunoblastic T-cell lymphoma. Antivir Ther 9:453–459
Brown HA, Macon WR, Kurtin PJ, Gibson LE (2001) Cutaneous involvement by angioimmunoblastic T-cell lymphoma with remarkable heterogeneous Epstein-Barr virus expression. J Cutan Pathol 28:432–438
Burke JS, Butler JJ (1976) Malignant lymphoma with a high content of epithelioid histiocytes (Lennert’s lymphoma). Am J Clin Pathol 66:1–9
Cadranel JF, Cadranel J, Buffet C, Fabre M, Pelletier G, d’Agay MF, Ink O, Roche A, Milleron B, Etienne JP (1990) Nodular regenerative hyperplasia of the liver, peliosis hepatis, and perisinusoidal fibrosis. Association with angioimmunoblastic lymphadenopathy and sever hypoxemia. Gastroenterology 99:268–273
Caulet S, Audouin J, Le Tourneau A, Diebold J, Bernardou A, Zittoun R (1988) Angio-immunoblastic (AIL) or T-cell malignant lymphoma of AIL-type. A histopathological, immunohistochemical and ultrastructural study of 8 cases. Pathol Res Pract 183:724–734
Chihara T, Wada N, Kohara M, Matsui T, Masaya H, Maeda T, Shibayama H, Kanakura Y et al (2010) Peripheral T-cell lymphoma of Lennert type complicated by monoclonal proliferation of large B-cells. Pathol Res Pract 206:185–190
Chu PG, Chang KL, Weiss LM, Arber DA (2000) Immunohistochemical detection of CD10 in paraffin sections of hematopoietic neoplasms: a comparison with flow cytometry detection in 56 cases. Appl Immunohistochem Mol Morphol 8:257–262
Cullen MH, Stansfeld AG, Oliver RT, Lister TA, Malpas JS (1979) Angio-immunoblastic lymphadenopathy: report of ten cases and review of the literature. Q J Med 48:151–177
Cutrona G, Leanza N, Ulivi M, Melioli G, Burgio VL, Mazzarelo G, Gabutti G, Roncella S et al (1999) Expression of CD10 by human T cells that undergo apoptosis both in vitro and in vivo. Blood 94:3067–3076
Diebold J, Tulliez M, Audouin J, Tricot G, Monsus M, Vilde F, Dubrisay J, Patri B, Arkwright S (1980) Visceral Kaposi sarcoma with angioimmunoblastic lymphadenopathy and latent sicca syndrome (in French). Bull Cancer 67:131–138
Dogan A, Attygalle AD, Kyriakou C (2003) Angioimmunoblastic T-cell lymphoma. Br J Haematol 121:681–691
Duvoux C, Kracht M, Lang P, Vernant JP, Zafrani ES, Dhumeaux D (1991) Nodular regenerative hyperplasia of the liver associated with azathioprine therapy (in French). Gastroenterol Clin Biol 15:968–973
Federico M, Rudiger T, Bellei M, Nathwani BN, Luminari S, Coiffier B, Harris NL, Jaffe ES et al (2013) Clinicopathologic characteristics of angioimmunoblastic T-cell lymphoma: analysis of the International Peripheral T-Cll Lymphoma Project. J Clin Oncol 31:240–246
Feller AC, Griesser GH, Mak TW, Lennert K (1986) Lymphoepithelioid lymphoma (Lennert’s lymphoma) is a monoclonal proliferation of helper/inducer T cells. Blood 68:663–667
Feller AC, Griesser H, Schilling CV, Wacker HH, Dallenbach F, Bartels H, Kuse R, Mak TW, Lennert K (1988) Clonal gene rearrangement patterns correlate with immunophenotype and clinical parameters in patients with angioimmunoblastic lymphadenopathy. Am J Pathol 133:549–556
Ferry JA (2002) Angioimmunoblastic T-cell lymphoma. Adv Anat Pathol 9:273–279
Franke WW, Moll R (1987) Cytoskeletal components of lymphoid organs. I. Synthesis of cytokeratins 8 and 18 and desmin in subpopulations of extrafollicular reticulum cells of human lymph nodes, tonsils, and spleen. Differentiation 36:145–163
Friedman-Birnbaum R, Gilhar A, Carter A (1985) Coexistence of Kaposi’s sarcoma and angioimmunoblastic lymphadenopathy. J Dermatol Surg Oncol 11:76–79
Frizzera G, Moran EM, Rappaport H (1974) Angio-immunoblastic lymphadenopathy with dysproteinemia. Lancet 866:1070–1073
Frizzera G, Moran EM, Rappaport H (1975) Angio-immunoblastic lymphadenopathy. Diagnosis and clinical course. Am J Med 59:803–818
Ganesan TS, Dhaliwal HS, Dorreen MS, Stansfeld AG, Habeshaw JA, Lister TA (1987) Angio-immunoblastic lymphadenopathy: a clinical, immunological and molecular study. Br J Cancer 55:437–442
Ghani AM, Krause JR (1985) Bone marrow biopsy findings in angioimmunoblastic lymphadenopathy. Br J Haematol 61:203–213
Gould VE, Bloom KJ, Franke WW, Warren WH, Moll R (1995) Increased numbers of cytokeratin-positive interstitial reticulum cells (CIRC) in reactive, inflammatory and neoplastic lymphadenopathies: hyperplasia or induced expression? Virchows Arch 425:617–629
Grogg KL, Attygalle AD, Macon WR, Remstein ED, Kurtin PJ, Dogan A (2006) Expression of CXCL13, a chemokine highly upregulated in germinal center T-helper cells, distinguishes angioimmunoblastic T-cell lymphoma from peripheral T-cell lymphoma, unspecified. Mod Pathol 19:1101–1107
Hartmann S, Agostinelli C, Klapper W, Korkolopoulou P, Koch K, Marafioti T, Piccaluga PP et al (2011) Revising the historical collection of epithelioid cell-rich lymphomas of the Kiel Lymph Node Registry: what is Lennert’s lymphoma nowadays? Histopathology 59:1173–1182
Higuchi T, Mori H, Niikura H, Omine M (1996) Hypocomplementemia and hematological abnormalities in immunoblastic lymphadenopathy and immunoblastic lymphadenopathy-like T cell lymphoma. Acta Haematol 96:68–72
Jones D Jorgensen JL, Shahsafaei A, Dorfman DM (1998) Characteristic proliferations of reticular and dendritic cells in angioimmunoblastic lymphoma. Am J Surg Pathol 22:956-964 (PubMed PMID 9706975)
Kitamura A, Yamashita Y, Sato Y, Hasegawa Y, Kojima H, Nagasawa T, Mori N (2005) Aggressive Lennert’s lymphoma: report of three cases in comparison to non-aggressive Lennert’s lymphoma. Pathol Int 55:626–631
Klapper W, Böhm M, Siebert R, Lennert K (2008) Morphological variability of lymphohistiocytic variant of anaplastic large cell lymphoma (former lymphohistiocytic lymphoma according to the Kiel classification). Virchows Arch 452:599–605
Knecht H, Schwarze EW, Lennert K (1985) Histological, immunohistological and autopsy findings in lymphogranulomatosis X (including angio-immunoblastic lymphadenopathy). Virchows Arch 406:105–124
Knudsen AH (2002) Peliosis hepatis as the cause of liver affection in a patient treated with immunosuppressive agents (in Danish). Ugeskr Laeger 164:3215–3216
Kojima M, Nakamura S, Itoh H, Motoori T, Sugihara S, Shinkai H, Masawa N (2001) Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centers: a clinicopathological and immunohistochemical study of 10 cases. APMIS 109:699–706
Kojima H, Shimizu S, Yoshida C, Katsura Y, Suzukawa K, Mukai HY, Hasegawa Y et al (2003) Rapidly progressive Lennert’s lymphoma terminating in fulminant hepatic failure. Leuk Lymphoma 44:1409–1412
Larrey D, Freneaux E, Berson A, Babany G, Degott C, Valla D, Pessayre, Benhamou JP (1988) Peliosis hepatis induced by 6-thioguanine administration. Gut 29:1265–1269
Lee PS, Lin CN, Chuang SS (2003a) Immunophenotyping of angioimmunoblastic T-cell lymphomas by multiparameter flow cytometry. Pathol Res Pract 199:539–545
Lee SS, Rudiger T, Odenwald T, Roth S, Starostik P, Müller-Hermelink HK (2003b) Angioimmunoblastic T cell lymphoma is derived from mature T-helper cells with varying expression and loss of detectable CD4. Int J Cancer 103:12–20
Lennert K (1973) Pathologisch-histologische Klassifizierung der malignen Lymphoma. In: Stacher A (ed) Leukämien und maligne Lymphoma. Urban and Schwarzenberg, München, pp 181–194
Lennert, Mestdagh (1968) Pubmed PMID 4972336
Lennert K, Mohri N (1974) Histologische Klassifizierung und Vorkommen des M. Hodgkin. Internist 15:57–65
Lennert K, Knecht H, Burkert M (1979) Vorstadien maligner Lymphome. Verh Dtsch Ges Pathol 63:170–196
Leung CY, Ho FC, Srivastava G, Loke SL, Liu YT, Chan AC (1993) Usefulness of follicular dendritic cell pattern in classification of peripheral T-cell lymphomas. Histopathology 23:433–437
Lome-Maldonado C, Canioni D, Hermine O, Delabesse E, Damotte D, Raffoux E, Gaulard P, Macintyre E et al (2002) Angio-immunoblastic T cell lymphoma (AILD-TL) rich in large B cells and associated with Epstein-Barr virus infection. A different subtype of AILD-TL? Leukemia 16:2134–2141
Lorcerie B, Grobost O, Lalu-Fraisse A, Piard F, Camus P, Portier H, Martin F (1990) Peliosis hepatis in dermatomyositis treated with azathioprine and corticoids (in French). Rev Med Interne 11:25–28
Lukes, Tindle (1975) Pubmed PMID 1078547
MacGillivray JB, Macintosh WG (1978) A case of Lennert’s lymphoma. J Clin Pathol 31:560–566
Molina Boix M, Ortega Gonzalez G, Perez Rigal V, Navarrete Montoya A, Lozano Garcia J, Alonso Lajara JD (1984) Vasculitis and cryoglobulinemia associated with an angioimmunoblastic lymphadenopathy. Study of 2 cases (in Spanish). Rev Clin Esp 175:117–118
Murakami T, Ohtsuki M, Nakagawa H (2001) Angioimmunoblastic lymphadenopathy-type peripheral T-cell lymphoma with cutaneous infiltration: report of a case and its gene expression profile. Br J Dermatol 144:878–884
Niitsu N, Nakamine H, Okamoto M, Akamatsu H, Honma Y, Higashihara M, Okabe-Kado J, Hirano M, Adult Lymphoma Treatment Study Group, ALTSG (2003) Expression of nm23-H1 is associated with poor prognosis in peripheral T-cell lymphoma. Br J Haematol 123:621–630
Ortonne N, Dupuis J, Plonquet A, Martin N, Copie-Bergman C, Bagot M, Delfau-Larue MH et al (2007) Characterization of CXCL13+ neoplastic T cells in cutaneous lesions of angioimmunoblastic T-cell lymphoma (AITL). Am J Surg Pathol 31:1068–1076
Palomero T, Couronné L, Khiabanian H, Kim MY, Ambesi-Impiombato A, Pereze-Garcia A et al (2014) Recurrent mutations in epigenetic regulators, RHO and FYN kinase in peripheral T cell lymphomas. Nat Genet 46:166–170
Pangalis GA, Moran EM, Rappaport H (1978) Blood and bone marrow findings in angioimmunoblastic lymphadenopathy. Blood 51:71–83
Patsouris E, Noel H, Lennert K (1988) Histological and immunohistological findings in lymphoepithelioid cell lymphoma (Lennert’s lymphoma). Am J Surg Pathol 12:341–350
Patsouris E, Engelhard M, Zwingers T, Lennert K (1993) Lymphoepithelioid cell lymphoma (Lennert’s lymphoma): clinical features derived from analysis of 108 cases. Br J Haematol 84:346–348
Radaszkiewicz T, Lennert K (1975) Lymphogranulomatosis X (immunoblastic adenopathy): clinical features, treatment and prognosis. Dtsch Med Wschr 21:1157–1163
Ree HJ, Kadin ME, Kikuchi M, Ko YH, Go JH, Suzumiya J, Kim DS (1998) Angioimmunoblastic lymphoma (AILD-type T-cell lymphoma) with hyperplastic germinal centers. Am J Surg Pathol 22:643–655
Ree HJ, Kadin ME, Kikuchi M, Ko YH, Suzumiya J, Go JH (1999) Bcl-6 expression in reactive follicular hyperplasia, follicular lymphoma, and angioimmunoblastic T-cell lymphoma with hyperplastic germinal centers: heterogeneity of intrafollicular T-cells and their altered distribution in the pathogenesis of angioimmunoblastic T-cell lymphoma. Hum Pathol 30:403–411
Reichard KK, Schwartz EJ, Higgins JP, Narasimhan B, Warnke RA, Natkunam Y (2006) CD10 expression in peripheral T-cell lymphomas complicated by a proliferation of large B-cells. Mod Pathol 19:337–343
Ren YL, Hong L, Nong L, Zhang S, Li T (2008) Clinicopathologic, immunohistochemical and molecular analysis in 15 cases of angioimmunoblastic T-cell lymphomas (in Chinese). Beijing Da Xue Xue Bao 40:352–357
Rodriguez-Justo M, Attygalle AD, Munson P, Roncador G, Marafioti T, Piris MA (2009) Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centres: a neoplasia with origin in the outer zone of the germinal centre? Clinicopathological and immunohistochemical study of 10 cases with follicular T-cell markers. Mod Pathol 22:753–761
Rubinstein A, Dauber LG (1983) Lymphoma of cytotoxic/suppressor T cell phenotype (T8) following angioimmunoblastic lymphadenopathy. Oncology 40:195–199
Sakata-Yanagimoto M, Enami T, Yoshida K, Shiraishi Y, Ishii R, Miyake Y, Muto H, Tsuyama N et al (2014) Somatic RHOA mutation in angioblastic T cell lymphoma. Nat Genet 46:171–175
Sallah S, Gagnon GA (1998) Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment. Acta Haematol 99:57–64
Sato I, Miura A, Yokomichi H, Suzuki C, Ichinohazama R (1990) Angio-immunoblastic lymphadenopathy with fibrosis of bone marrow, lymph node, liver and spleen, and proliferation of epithelioid cells in lymph nodes (in Japanese). Rinsho Ketsueki 31:958–962
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA (2011) Peripheral T-cell lymphoma – not otherwise specified. Crit Rev Oncol Hematol 79:321–329
Schauer PK, Straus DJ, Bagley CM, Rudolph RH, McCracken JD, Huff J, Glucksburg H, Bauermeister DE, Clarkson BD (1981) Angioimmunoblastic lymphadenopathy: clinical spectrum of disease. Cancer 48:2493–2498
Seehafer JR, Goldberg NC, Dicken CH, Su WP (1980) Cutaneous manifestations of angioimmunoblastic lymphadenopathy. Arch Dermatol 116:41–45
Shin SY, Jang S, Park CJ, Chi HS, Lee KH, Huh J, Seo EJ (2012) A rare case of Lennert’s type peripheral T-cell lymphoma with t(14;19)(q11.2;q13.3). Int J Lab Hematol 34:328–332
Simon G, Faller JP, Carbillet JP (1983) Association of angioimmunoblastic lymphadenopathy and vasculitis (in French). Presse Med 12:2461–2462
Simon et al. (1988) Pubmed PMID 3135227
Staples WG, Gétaz EP (1977) Lympho-epithelioid cellular lymphoma (Lennert’s lymphoma): a case report. S Afr Med J 51:555–556
Sugaya et al. (2001) Pubmed PMID 11280462
Suster S, Ronnen M, Bubis JJ (1987) Angioimmunoblastic lymphadenopathy following Kaposi’s sarcoma. Am J Med Sci 294:249–252
Takai K, Sanada M (2000) Angioimmunoblastic T-cell lymphoma associated with hemophagocytic syndrome at onset and relapse (in Japanese). Rinsho Ketsueki 41:1260–1266
Warlow R, Vernea S, Mijch A (1984) Angioimmunoblastic lymphadenopathy associated with large-muscle arteritis. Hum Pathol 15:486–490
Wegerle W, Garbrecht M, Nerl C, Schmitt W (1994) Angioimmunoblastic lymphadenopathy with dysproteinemia and sclerosing cholangitis (in German). Dtsch Med Woschenschr 119:332–337
Willenbrock K, Bräuninger A, Hansmann ML (2007) 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 138:733–739
Yoo HY, Sung MK, Lee SH, Kim S, Lee H, Park S, Kim SC, Lee B, Rho K, Lee JE et al (2014) A recurrent inactivating mutation in RHOA GTPase in angioblastic T cell lymphoma. Nat Genet 46:371–375
Zettl A, Lee SS, Rudiger T, Starostik P, Marino M, Kirchner T, Ott M, Müller-Hermelink HK (2002) Epstein-Barr virus-associated B-cell lymphoproliferative disorders in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, unspecified. Am J Clin Pathol 117:386–379
Zhao WL, Mourah S, Mounier N, Leboef C, Daneshpouy ME, Legres L, Meignin V, Oksenhendler E et al (2004) Vascular endothelial growth factor-A is expressed both on lymphoma cells and endothelial cells in angioimmunoblastic T-cell lymphoma and related to lymphoma progression. Lab Invest 84:1512–1519
Zhou Y, Attygalle AD, Chuang SS, Diss T, Ye H, Liu H, Hamoudi RA, Munson P et al (2007) Angioimmunoblastic T-cell lymphoma: histological progression associated with EBV and HHV6B viral load. Br J Haematol 138:44–53
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Zimmermann, A. (2017). T-Cell Non-Hodgkin’s Lymphomas: Variant Forms. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_96
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