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Enteropathy-Associated T-Cell Lymphomas

  • Stephanie Teja
  • Neha Mehta-ShahEmail author
Chapter

Abstract

Enteropathy-associated T-cell lymphoma (EATL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) are two rare forms of T-cell lymphoma that primarily present in the intestine and were previously collectively termed EATL. Given their distinct immunohistochemistry, mutational profile, and epidemiology, they were formally separated in 2016. Many patients present with an acute abdomen and are diagnosed during laparotomy. While outcomes are significantly better for those who receive anthracycline-based chemotherapy, some patients are not candidates for systemic therapy and have particularly poor outcomes. There is no standard approach to the management of EATL and MEITL. Novel therapies hold promise for the future.

Keywords

Enteropathy-associated T-cell lymphoma Monomorphic epitheliotropic intestinal T-cell lymphoma Enteropathy-associated T-cell lymphoma type I Enteropathy-associated T-cell lymphoma type II EATL MEITL 

References

  1. 1.
    Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90.PubMedPubMedCentralCrossRefGoogle Scholar
  2. 2.
    Ferreri AJM, Zinzani PL, Govi S, Pileri SA. Enteropathy-associated T-cell lymphoma. Crit Rev Oncol Hematol. 2011;79(1):84–90.PubMedCrossRefPubMedCentralGoogle Scholar
  3. 3.
    Tse E, Gill H, Loong F, Kim SJ, Ng SB, Tang T, et al. Type II enteropathy-associated T-cell lymphoma: a multicenter analysis from the Asia Lymphoma Study Group. Am J Hematol. 2012;87(7):663–8.PubMedCrossRefPubMedCentralGoogle Scholar
  4. 4.
    Deleeuw RJ, Zettl A, Klinker E, Haralambieva E, Trottier M, Chari R, et al. Whole-genome analysis and HLA genotyping of enteropathy-type T-cell lymphoma reveals 2 distinct lymphoma subtypes. Gastroenterology. 2007;132(5):1902–11.PubMedCrossRefGoogle Scholar
  5. 5.
    Delabie J, Holte H, Vose JM, Ullrich F, Jaffe ES, Savage KJ, et al. Enteropathy-associated T-cell lymphoma: clinical and histological findings from the international peripheral T-cell lymphoma project. Blood. 2011;118(1):148–55.CrossRefGoogle Scholar
  6. 6.
    Gale J, Simmonds PD, Mead GM, Sweetenham JW, Wright DH. Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center. J Clin Oncol. 2000;18(4):795–803.PubMedCrossRefGoogle Scholar
  7. 7.
    Sieniawski M, Angamuthu N, Boyd K, Chasty R, Davies J, Forsyth P, et al. Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation. Blood. 2010;115(18):3664–70.PubMedCrossRefGoogle Scholar
  8. 8.
    Verbeek WHM, Van De Water JMW, Al-Toma A, Oudejans JJ, Mulder CJJ, Coupe VMH. Incidence of enteropathy - associated T-cell lymphoma: a nation-wide study of a population-based registry in the Netherlands. Scand J Gastroenterol. 2008;43(11):1322–8.PubMedCrossRefPubMedCentralGoogle Scholar
  9. 9.
    Lee MY, Tsou MH, Tan TD, Lu MC. Clinicopathological analysis of T-cell lymphoma in Taiwan according to WHO classification: high incidence of enteropathy-type intestinal T-cell lymphoma. Eur J Haematol. 2005;75(3):221–6.PubMedCrossRefGoogle Scholar
  10. 10.
    Chan JKC, Chan ACL, Cheuk W, Wan SK, Lee WK, Lui YH, et al. Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent gamma delta T-cell receptor expression. Am J Surg Pathol. 2011;35(10):1557–69.PubMedCrossRefPubMedCentralGoogle Scholar
  11. 11.
    Sun J, Lu ZH, Yang D, Chen J. Primary intestinal T-cell and NK-cell lymphomas: a clinicopathological and molecular study from China focused on type II enteropathy-associated T-cell lymphoma and primary intestinal NK-cell lymphoma. Mod Pathol. 2011;24(7):983–92.PubMedCrossRefPubMedCentralGoogle Scholar
  12. 12.
    Takeshita M, Nakamura S, Kikuma K, Nakayama Y, Nimura S, Yao T, et al. Pathological and immunohistological findings and genetic aberrations of intestinal enteropathy-associated T cell lymphoma in Japan. Histopathology. 2011;58(3):395–407.PubMedCrossRefPubMedCentralGoogle Scholar
  13. 13.
    Karanam PK, Al-Hamadani M, Go RS. Enteropathy-associated T-cell lymphoma in the US: higher incidence and poorer survival among Asians. Br J Haematol. 2016;172(6):990–2.PubMedCrossRefPubMedCentralGoogle Scholar
  14. 14.
    Ilyas M, Niedobitek G, Agathanggelou A, Barry RE, Read AE, Tierney R, et al. Non-Hodgkins-lymphoma, celiac-disease, and Epstein-Barr-virus – a study of 13 cases of enteropathy-associated t-cell and b-cell lymphoma. J Pathol. 1995;177(2):115–22.PubMedCrossRefPubMedCentralGoogle Scholar
  15. 15.
    Di Sabatino A, Biagi F, Gobbi PG, Corazza GR. How I treat enteropathy-associated T-cell lymphoma. Blood. 2012;119(11):2458–68.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    Silano M, Volta U, De Vincenzi A, Dessi M, De Vincenzi M, Collaborating Centers of the Italian Registry of the Complications of Coeliac Disease. Effect of a gluten-free diet on the risk of enteropathy-associated T-cell lymphoma in celiac disease. Dig Dis Sci. 2008;53(4):972–6.PubMedCrossRefPubMedCentralGoogle Scholar
  17. 17.
    Green PHR, Fleischauer AT, Bhagat G, Goyal R, Jabri B, Neugut AI. Risk of malignancy in patients with celiac disease. Am J Med. 2003;115(3):191–5.PubMedCrossRefPubMedCentralGoogle Scholar
  18. 18.
    Sieniawski MK, Lennard AL. Enteropathy-associated T-cell lymphoma: epidemiology, clinical features, and current treatment strategies. Curr Hematol Malig Rep. 2011;6(4):231–40.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Nijeboer P, de Baaij LR, Visser O, Witte BI, Cillessen SA, Mulder CJ, et al. Treatment response in enteropathy associated T-cell lymphoma; survival in a large multicenter cohort. Am J Hematol. 2015;90(6):493–8.PubMedCrossRefPubMedCentralGoogle Scholar
  20. 20.
    de Baaij LR, Berkhof J, van de Water JM, Sieniawski MK, Radersma M, Verbeek WH, et al. A new and validated clinical prognostic model (EPI) for enteropathy-associated T-cell lymphoma. Clin Cancer Res. 2015;21(13):3013–9.PubMedCrossRefPubMedCentralGoogle Scholar
  21. 21.
    Wierdsma NJ, Nijeboer P, de van der Schueren MAE, Berkenpas M, van Bodegraven AA, Mulder CJJ. Refractory celiac disease and EATL patients show severe malnutrition and malabsorption at diagnosis. Clin Nutr. 2016;35(3):685–91.PubMedCrossRefPubMedCentralGoogle Scholar
  22. 22.
    Amiot A, Allez M, Treton X, Fieschi C, Galicier L, Joly F, et al. High frequency of fatal haemophagocytic lymphohistiocytosis syndrome in enteropathy-associated T cell lymphoma. Dig Liver Dis. 2012;44(4):343–9.PubMedCrossRefPubMedCentralGoogle Scholar
  23. 23.
    Malamut G, Chandesris O, Verkarre V, Meresse B, Callens C, Macintyre E, et al. Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. Dig Liver Dis. 2013;45(5):377–84.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Lu L, Ning SY, Kassam Z, Puglia M. Gluten gambit: a case of Hemophagocytic Lymphohistiocytic syndrome and enteropathy-associated T-cell lymphoma in a patient with refractory celiac disease. Am J Gastroenterol. 2012;107:S373–S4.CrossRefGoogle Scholar
  25. 25.
    Varghese D, Koya HH, Cherian SV, Mead K, Sharma A, Sharma N, et al. Hemophagocytic lymphohistiocytosis: an uncommon presentation of enteropathy-associated T-cell lymphoma. J Clin Oncol. 2013;31(13):E226–E30.PubMedCrossRefPubMedCentralGoogle Scholar
  26. 26.
    Berman EL, Zauber NP, Rickert RR, Diss TC, Isaacson PG. Enteropathy-associated T cell lymphoma with brain involvement. J Clin Gastroenterol. 1998;26(4):337–41.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Gobbi C, Buess M, Probst A, Ruegg S, Schraml P, Herrmann R, et al. Enteropathy-associated T-cell lymphoma with initial manifestation in the CNS. Neurology. 2003;60(10):1718–9.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Bewig B, Wacker HH, Parwaresch MR, Nitsche R, Folsch UR. Eosinophilia-leading symptom of an enteropathy-associated T-cell lymphoma of high malignancy. Z Gastroenterol. 1993;31(11):666–70.PubMedPubMedCentralGoogle Scholar
  29. 29.
    Hovenga S, de Graaf H, Joosten P, van den Berg GA, Storm H, Langerak AW, et al. Enteropathy-associated T-cell lymphoma presenting with eosinophilia. Neth J Med. 2003;61(1):25–7.PubMedPubMedCentralGoogle Scholar
  30. 30.
    Soon CW, Kirsch IR, Connolly AJ, Kwong BY, Kim J. Eosinophil-rich acute febrile neutrophilic dermatosis in a patient with enteropathy-associated T-cell lymphoma, type 1. Am J Dermatopathol. 2016;38(9):704–8.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Schwock J, Hyjek EM, Torlakovic EE, Geddie WR. Enteropathy-associated intestinal T-cell lymphoma in cavitating mesenteric lymph node syndrome: fine-needle aspiration contributes to the diagnosis. Diagn Cytopathol. 2015;43(2):125–30.PubMedCrossRefPubMedCentralGoogle Scholar
  32. 32.
    Jousserand G, Poujois A, Antoine JC, Camdessanché JP. [Polyradiculopathy revealing an enteropathy associated T-cell lymphoma in a patient with celiac disease]. Rev Neurol (Paris). 2009;165(1):89–91.Google Scholar
  33. 33.
    Chan T, Lee E, Khong PL, Kwong YL, Tse E. Positron emission tomography computed tomography features of type ii enteropathy associated t cell lymphoma. Haematologica. 2016;101:689.Google Scholar
  34. 34.
    Mudhar HS, Fernando M, Rennie IG, Evans LS. Enteropathy-associated T-cell lymphoma, lacking MHC class II, with immune-privileged site recurrence, presenting as bilateral ocular vitreous humour involvement – a case report. Histopathology. 2012;61(6):1227–30.PubMedCrossRefGoogle Scholar
  35. 35.
    Antoniadou F, Dimitrakopoulou A, Voutsinas PM, Vrettou K, Vlahadami I, Voulgarelis M, et al. Monomorphic epitheliotropic intestinal T-cell lymphoma in pleural effusion: a case report. Diagn Cytopathol. 2017;45(11):1050–4.PubMedCrossRefGoogle Scholar
  36. 36.
    Tanaka H, Ambiru S, Nakamura S, Itabashi T, Furuya S, Shimura T, et al. Successful diagnosis of type II enteropathy-associated T-cell lymphoma using flow cytometry and the cell block technique of celomic fluid manifesting as massive pyoid ascites that could not be diagnosed via emergency laparotomy. Intern Med. 2014;53(2):129–33.PubMedCrossRefGoogle Scholar
  37. 37.
    Webster A, Crea P, Bamford MW, Hew R, Griffin Y, Miall F. Enteropathy-associated T-cell lymphoma presenting as cutaneous deposits. Br J Haematol. 2017;176(1):7.PubMedCrossRefGoogle Scholar
  38. 38.
    Chen JH, Bai D, Dabhi V, Wood BL, Kussick SJ. Clinicopathologic features of primary colonic enteropathy-associated T cell lymphoma type II in an elderly Asian male with diarrhea. J Hematop. 2015;8(1):37–41.CrossRefGoogle Scholar
  39. 39.
    Tomizawa Y, Van Slambrouck C, Kavitt RT. A rare cause of diarrhea and weight loss type II (monomorphic) enteropathy-associated T-cell lymphoma. Gastroenterology. 2015;148(7):1288–9.PubMedCrossRefGoogle Scholar
  40. 40.
    Kaif M, Fitzmorris P, Weber F. A rare case of chronic diarrhea enteropathy-associated T-cell lymphoma, type II. Gastroenterology. 2015;148(3):510–2.PubMedCrossRefPubMedCentralGoogle Scholar
  41. 41.
    Jacob PM, Nair RA, Mehta J, Borges AM, Suchetha S. Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses. Indian J Pathol Microbiol. 2014;57(2):326–8.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    Chott A, Vesely M, Simonitsch I, Mosberger I, Hanak H. Classification of intestinal T-cell neoplasms and their differential diagnosis. Am J Clin Pathol. 1999;111(1):S68–74.PubMedPubMedCentralGoogle Scholar
  43. 43.
    Smedby KE, Akerman M, Hildebrand H, Glimelius B, Ekbom A, Askling J. Malignant lymphomas in coeliac disease: evidence of increased risks for lymphoma types other than enteropathy-type T cell lymphoma. Gut. 2005;54(1):54–9.PubMedPubMedCentralCrossRefGoogle Scholar
  44. 44.
    Perry AM, Warnke RA, Hu QL, Gaulard P, Copie-Bergman C, Alkan S, et al. Indolent T-cell lymphoproliferative disease of the gastrointestinal tract. Blood. 2013;122(22):3599–606.PubMedPubMedCentralCrossRefGoogle Scholar
  45. 45.
    Arps DP, Smith LB. Classic versus type II enteropathy-associated T-cell lymphoma: diagnostic considerations. Arch Pathol Lab Med. 2013;137(9):1227–31.PubMedCrossRefPubMedCentralGoogle Scholar
  46. 46.
    Mutzbauer G, Maurus K, Buszello C, Pischimarov J, Roth S, Rosenwald A, et al. SYK expression in monomorphic epitheliotropic intestinal T-cell lymphoma. Mod Pathol. 2018;31(3):505–16.PubMedCrossRefGoogle Scholar
  47. 47.
    Roberti A, Dobay MP, Bisig B, Vallois D, Boéchat C, Lanitis E, et al. Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations. Nat Commun. 2016;7:12602.PubMedPubMedCentralCrossRefGoogle Scholar
  48. 48.
    Nairismagi ML, Tan J, Lim JQ, Nagarajan S, Ng CCY, Rajasegaran V, et al. JAK-STAT and G-protein-coupled receptor signaling pathways are frequently altered in epitheliotropic intestinal T-cell lymphoma. Leukemia. 2016;30(6):1311–9.PubMedPubMedCentralCrossRefGoogle Scholar
  49. 49.
    Moffitt AB, Ondrejka SL, McKinney M, Rempel RE, Goodlad JR, Teh CH, et al. Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2. J Exp Med. 2017;214(5):1371–86.PubMedPubMedCentralCrossRefGoogle Scholar
  50. 50.
    Hadithi M, Mallant M, Oudejans J, van Waesberghe J, Mulder CJ, Comans EFI. F-18-FDG PET versus CT for the detection of enteropathy-associated T-cell lymphoma in refractory celiac disease. J Nucl Med. 2006;47(10):1622–7.PubMedPubMedCentralGoogle Scholar
  51. 51.
    Ellin F, Landstrom J, Jerkeman M, Relander T. Real-world data on prognostic factors and treatment in peripheral T-cell lymphomas: a study from the Swedish Lymphoma Registry. Blood. 2014;124(10):1570–7.PubMedCrossRefPubMedCentralGoogle Scholar
  52. 52.
    Mehta-Shah N, Horwitz S. Zebras and hen’s teeth: recognition and management of rare T and NK lymphomas. Hematology Am Soc Hematol Educ Program. 2015;2015:545–9.PubMedCrossRefPubMedCentralGoogle Scholar
  53. 53.
    Novakovic BJ, Novakovic S, Frkovic-Grazio S. A single-center report on clinical features and treatment response in patients with intestinal T cell non-Hodgkin’s lymphomas. Oncol Rep. 2006;16(1):191–5.PubMedPubMedCentralGoogle Scholar
  54. 54.
    Egan LJ, Walsh SV, Stevens FM, Connolly CE, Egan EL, McCarthy CF. Celiac-associated lymphoma – a single institution experience of 30 cases in the combination chemotherapy era. J Clin Gastroenterol. 1995;21(2):123–9.PubMedCrossRefPubMedCentralGoogle Scholar
  55. 55.
    Honemann D, Prince HM, Hicks RJ, Seymour JF. Enteropathy-associated T-cell lymphoma without a prior diagnosis of coeliac disease: diagnostic dilemmas and management options. Ann Hematol. 2005;84(2):118–21.PubMedCrossRefPubMedCentralGoogle Scholar
  56. 56.
    Wohrer S, Chott A, Drach J, Puspok A, Hejna M, Hoffmann M, et al. Chemotherapy with cyclophosphamide, doxorubicin, etoposide, vincristine and prednisone (CHOEP) is not effective in patients with enteropathy-type intestinal T-cell lymphoma. Ann Oncol. 2004;15(11):1680–3.PubMedCrossRefPubMedCentralGoogle Scholar
  57. 57.
    Daum S, Ullrich R, Heise W, Dederke B, Foss HD, Stein H, et al. Intestinal non-Hodgkin’s lymphoma: a multicenter prospective clinical study from the German Study Group on Intestinal non-Hodgkin’s Lymphoma. J Clin Oncol. 2003;21(14):2740–6.PubMedCrossRefPubMedCentralGoogle Scholar
  58. 58.
    d’Amore F, Relander T, Lauritzsen GF, Jantunen E, Hagberg H, Anderson H, et al. Up-front autologous stem-cell transplantation in peripheral T-cell lymphoma: NLG-T-01. J Clin Oncol. 2012;30(25):3093–9.PubMedCrossRefPubMedCentralGoogle Scholar
  59. 59.
    Bishton MJ, Haynes AP. Combination chemotherapy followed by autologous stem cell transplant for enteropathy-associated T cell lymphoma. Br J Haematol. 2007;136(1):111–3.PubMedCrossRefPubMedCentralGoogle Scholar
  60. 60.
    Jantunen E, Boumendil A, Finel H, Luan JJ, Johnson P, Rambaldi A, et al. Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT. Blood. 2013;121(13):2529–32.PubMedCrossRefPubMedCentralGoogle Scholar
  61. 61.
    Nijeboer P, Malamut G, Mulder CJ, Cerf-Bensussan N, Sibon D, Bouma G, et al. Enteropathy-associated T-cell lymphoma: improving treatment strategies. Dig Dis. 2015;33(2):231–5.PubMedCrossRefPubMedCentralGoogle Scholar
  62. 62.
    Ikebe T, Miyazaki Y, Abe Y, Urakami K, Ohtsuka E, Saburi Y, et al. Successful treatment of refractory enteropathy-associated T-cell lymphoma using high-dose chemotherapy and autologous stem cell transplantation. Intern Med. 2010;49(19):2157–61.PubMedCrossRefPubMedCentralGoogle Scholar
  63. 63.
    Raderer M, Troch M, Kiesewetter B, Puspok A, Jaeger U, Hoffmann M, et al. Second line chemotherapy in patients with enteropathy-associated T cell lymphoma: a retrospective single center analysis. Ann Hematol. 2012;91(1):57–61.PubMedCrossRefPubMedCentralGoogle Scholar
  64. 64.
    Mehta-Shah N, Teja ST, Tao Y, Cashen A, Beaven A, Alpdoogan O, et al. Successful treatment of mature T-cell lymphoma with allogeneic stem cell transplantation: the largest multicenter retrospective analysis. American Society of Hematology 59th Annual Meeting & Exposition; 12/11/2017; Atlanta, Georgia. 2017.Google Scholar
  65. 65.
    Foyil KV, Bartlett NL. Brentuximab vedotin for the treatment of CD30(+) lymphomas. Immunotherapy. 2011;3(4):475–85.PubMedCrossRefPubMedCentralGoogle Scholar
  66. 66.
    Khalaf WF, Caldwell ME, Reddy N. Brentuximab in the treatment of CD30-positive enteropathy-associated T-cell lymphoma. J Natl Compr Cancer Netw. 2013;11(2):137–40.CrossRefGoogle Scholar
  67. 67.
    Haslbauer F. Brentuximab vedotin in a patient with enteropathy-associated T-cell lymphoma of the small bowel. Memo-Mag Eur Med Oncol. 2017;10(2):111–4.CrossRefGoogle Scholar
  68. 68.
    Marquard L, Poulsen CB, Gjerdrum LM, Brown PD, Christensen IJ, Jensen PB, et al. Histone deacetylase 1, 2, 6 and acetylated histone H4 in B- and T-cell lymphomas. Histopathology. 2009;54(6):688–98.PubMedCrossRefPubMedCentralGoogle Scholar
  69. 69.
    Piekarz RL, Frye R, Prince HM, Kirschbaum MH, Zain J, Allen SL, et al. Phase 2 trial of romidepsin in patients with peripheral T-cell lymphoma. Blood. 2011;117(22):5827–34.PubMedPubMedCentralCrossRefGoogle Scholar
  70. 70.
    Zhang YP, Xu W, Liu H, Li JY. Therapeutic options in peripheral T cell lymphoma. J Hematol Oncol. 2016;9:37.PubMedPubMedCentralCrossRefGoogle Scholar
  71. 71.
    Iyer SP, Foss FF. Romidepsin for the treatment of peripheral T-cell lymphoma. Oncologist. 2015;20(9):1084–91.PubMedPubMedCentralCrossRefGoogle Scholar
  72. 72.
    O’Connor OA, Horwitz S, Masszi T, Van Hoof A, Brown P, Doorduijn J, et al. Belinostat in patients with relapsed or refractory peripheral T-cell lymphoma: results of the pivotal phase II BELIEF (CLN-19) study. J Clin Oncol. 2015;33(23):2492–U21.PubMedPubMedCentralCrossRefGoogle Scholar
  73. 73.
    Horwitz SM, Porcu P, Flinn I, Kahl BS, Sweeney J, Stern HM, et al. Duvelisib (IPI-145), a phosphoinositide-3-kinase-delta,gamma inhibitor, shows activity in patients with relapsed/refractory T-cell lymphoma. Blood. 2014;124(21):803.CrossRefGoogle Scholar
  74. 74.
    Porcu P, Flinn I, Kahl BS, Horwitz SM, Oki Y, Byrd JC, et al. Clinical activity of duvelisib (IPI-145), a phosphoinositide-3-kinase-delta, gamma inhibitor, in patients previously treated with ibrutinib. Blood. 2014;124(21):Abstract 3335.CrossRefGoogle Scholar
  75. 75.
    Horwitz SM, Koch R, Porcu P, Oki Y, Moskowitz A, Perez M, et al. Activity of the PI3K-delta,gamma inhibitor duvelisib in a phase 1 trial and preclinical models of T-cell lymphoma. Blood. 2018;131(8):888–98.PubMedPubMedCentralCrossRefGoogle Scholar
  76. 76.
    de Baaij LR, Radersma M, van de Water JM, Hijmering NJ, Moesbergen L, Visser O, et al. Bortezomib restores defective apoptosis by upregulation of Noxa in enteropathy-associated T-cell lymphoma. Gastroenterology. 2013;144(5):S250–S.CrossRefGoogle Scholar
  77. 77.
    Toumishey E, Prasad A, Dueck G, Chua N, Finch D, Johnston J, et al. Final report of a phase 2 clinical trial of lenalidomide monotherapy for patients with T-cell lymphoma. Cancer. 2015;121(5):716–23.PubMedCrossRefPubMedCentralGoogle Scholar
  78. 78.
    Rodig SJ, Abramson JS, Pinkus GS, Treon SP, Dorfman DM, Dong HY, et al. Heterogeneous CD52 expression among hematologic neoplasms: implications for the use of alemtuzumab (CAMPATH-1H). Clin Cancer Res. 2006;12(23):7174–9.PubMedCrossRefGoogle Scholar
  79. 79.
    Gallamini A, Zaja F, Patti C, Billio A, Specchia MR, Tucci A, et al. Alemtuzumab (Campath-1H) and CHOP chemotherapy as first-line treatment of peripheral T-cell lymphoma: results of a GITIL (Grappo Italiano Terapie Innovative nei Linfomi) prospective multicenter trial. Blood. 2007;110(7):2316–23.PubMedCrossRefGoogle Scholar
  80. 80.
    Soldini D, Mora O, Cavalli F, Zucca E, Mazzucchelli L. Efficacy of alemtuzumab and gemcitabine in a patient with enteropathy-type T-cell lymphoma. Br J Haematol. 2008;142(3):484–6.PubMedCrossRefPubMedCentralGoogle Scholar
  81. 81.
    Gentille C, Qin Q, Barbieri A, Ravi PS, Iyer S. Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma, a disease with diagnostic and therapeutic challenges. Ecancermedicalscience. 2017;11:771.PubMedPubMedCentralCrossRefGoogle Scholar
  82. 82.
    Hamlin P, Farber C, Fenske T, Khatcheressian J, Miller C, Munoz J, et al. The dual SYK/JAK inhibitor cerdulatinib demonstrates complete inhibition of SYK and JAK and rapid tumor responses in a phase 2 study in patients with relapsed/refractory b cell malignancies. Haematologica. 2017;102:312–3.Google Scholar
  83. 83.
    Feldman AL, Sun DX, Law ME, Novak AJ, Attygalle AD, Thorland EC, et al. Overexpression of Syk tyrosine kinase in peripheral T-cell lymphomas. Leukemia. 2008;22(6):1139–43.PubMedPubMedCentralCrossRefGoogle Scholar
  84. 84.
    Wilcox RA, Sun DX, Novak A, Dogan A, Ansell SM, Feldman AL. Inhibition of Syk protein tyrosine kinase induces apoptosis and blocks proliferation in T-cell non-Hodgkin’s lymphoma cell lines. Leukemia. 2010;24(1):229–32.PubMedCrossRefPubMedCentralGoogle Scholar

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Washington University School of Medicine in St. LouisSt. LouisUSA
  2. 2.Division of OncologyWashington University Medical SchoolSt. LouisUSA

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