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. 1992 Dec;141(6):1361–1371.

Peripheral T-cell lymphomas of the intestine.

A Chott 1, B Dragosics 1, T Radaszkiewicz 1
PMCID: PMC1886751  PMID: 1466400

Abstract

Twenty-seven cases of primary peripheral T-cell lymphomas of the intestine (PTLI) were investigated. Seven patients had histories of malabsorption. The most frequent symptoms at presentation were weight loss, abdominal pain, and acute abdomen. The jejunum was the most common site of lymphoma and multifocal disease was found in 72% of the cases. Twenty-two patients (92%) presented with localized disease confined to the intestine and abdominal lymph nodes, only two patients had generalized disease. According to the pattern of lymphoma infiltration and the morphology of the uninvolved small intestinal mucosa, 21 cases were separated histologically into three categories; 1) enteropathy-associated T-cell lymphoma (EATCL, n = 9) showing predominant intramucosal lymphoma spread and villous atrophy of uninvolved mucosa with high density of intraepithelial lymphocytes (IEL), 2) EATCL-like lymphoma without enteropathy (EATCL-LLWE, n = 5) but with an infiltration pattern similar to EATCL, and 3) T-cell lymphoma without features of EATCL (Non-EATCL, n = 7). Distinctive features of EATCL were the high incidence of malabsorption states, multifocal intestinal disease in all cases, and the high frequency of intestinal recurrences. On frozen sections four of eight PTLI showed the phenotype CD3+ CD4- CD8- HML-1+, which is also expressed on a small subset of normal IEL. The morphologic and immunomorphologic findings suggest that the majority of PTLI is derived from mucosal T lymphocytes. This derivation may be responsible for certain biologic features, such as the preferential spread to and relapse of PTLI at small intestinal sites.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Al-Mondhiry H. Primary lymphomas of the small intestine: east-west contrast. Am J Hematol. 1986 May;22(1):89–105. doi: 10.1002/ajh.2830220114. [DOI] [PubMed] [Google Scholar]
  2. Armitage J. O., Greer J. P., Levine A. M., Weisenburger D. D., Formenti S. C., Bast M., Conley S., Pierson J., Linder J., Cousar J. B. Peripheral T-cell lymphoma. Cancer. 1989 Jan 1;63(1):158–163. doi: 10.1002/1097-0142(19890101)63:1<158::aid-cncr2820630125>3.0.co;2-b. [DOI] [PubMed] [Google Scholar]
  3. Carbone P. P., Kaplan H. S., Musshoff K., Smithers D. W., Tubiana M. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res. 1971 Nov;31(11):1860–1861. [PubMed] [Google Scholar]
  4. Cerf-Bensussan N., Jarry A., Brousse N., Lisowska-Grospierre B., Guy-Grand D., Griscelli C. A monoclonal antibody (HML-1) defining a novel membrane molecule present on human intestinal lymphocytes. Eur J Immunol. 1987 Sep;17(9):1279–1285. doi: 10.1002/eji.1830170910. [DOI] [PubMed] [Google Scholar]
  5. Chan J. K., Ng C. S., Hui P. K., Leung T. W., Lo E. S., Lau W. H., McGuire L. J. Anaplastic large cell Ki-1 lymphoma. Delineation of two morphological types. Histopathology. 1989 Jul;15(1):11–34. doi: 10.1111/j.1365-2559.1989.tb03038.x. [DOI] [PubMed] [Google Scholar]
  6. Chott A., Augustin I., Wrba F., Hanak H., Ohlinger W., Radaszkiewicz T. Peripheral T-cell lymphomas: a clinicopathologic study of 75 cases. Hum Pathol. 1990 Nov;21(11):1117–1125. doi: 10.1016/0046-8177(90)90148-x. [DOI] [PubMed] [Google Scholar]
  7. Chott A., Kaserer K., Augustin I., Vesely M., Heinz R., Oehlinger W., Hanak H., Radaszkiewicz T. Ki-1-positive large cell lymphoma. A clinicopathologic study of 41 cases. Am J Surg Pathol. 1990 May;14(5):439–448. doi: 10.1097/00000478-199005000-00003. [DOI] [PubMed] [Google Scholar]
  8. Cooper B. T., Read A. E. Small intestinal lymphoma. World J Surg. 1985 Dec;9(6):930–937. doi: 10.1007/BF01655398. [DOI] [PubMed] [Google Scholar]
  9. Dragosics B., Bauer P., Radaszkiewicz T. Primary gastrointestinal non-Hodgkin's lymphomas. A retrospective clinicopathologic study of 150 cases. Cancer. 1985 Mar 1;55(5):1060–1073. doi: 10.1002/1097-0142(19850301)55:5<1060::aid-cncr2820550523>3.0.co;2-8. [DOI] [PubMed] [Google Scholar]
  10. Falini B., Flenghi L., Fagioli M., Pelicci P. G., Stein H., Bigerna B., Pileri S., Martelli M. F. Expression of the intestinal T-lymphocyte associated molecule HML-1: analysis of 75 non-Hodgkin's lymphomas and description of the first HML-1 positive T-lymphoblastic tumour. Histopathology. 1991 May;18(5):421–426. doi: 10.1111/j.1365-2559.1991.tb00872.x. [DOI] [PubMed] [Google Scholar]
  11. Ferguson A., Murray D. Quantitation of intraepithelial lymphocytes in human jejunum. Gut. 1971 Dec;12(12):988–994. doi: 10.1136/gut.12.12.988. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Foucar K., Foucar E., Mitros F., Clamon G., Goeken J., Crossett J. Epitheliotropic lymphoma of the small bowel. Report of a fatal case with cytotoxic/suppressor T-cell immunotype. Cancer. 1984 Jul 1;54(1):54–60. doi: 10.1002/1097-0142(19840701)54:1<54::aid-cncr2820540113>3.0.co;2-a. [DOI] [PubMed] [Google Scholar]
  13. Freeman C., Berg J. W., Cutler S. J. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972 Jan;29(1):252–260. doi: 10.1002/1097-0142(197201)29:1<252::aid-cncr2820290138>3.0.co;2-#. [DOI] [PubMed] [Google Scholar]
  14. Grody W. W., Magidson J. G., Weiss L. M., Hu E., Warnke H. A., Lewin K. J. Gastrointestinal lymphomas. Immunohistochemical studies on the cell of origin. Am J Surg Pathol. 1985 May;9(5):328–337. [PubMed] [Google Scholar]
  15. Horning S. J., Weiss L. M., Crabtree G. S., Warnke R. A. Clinical and phenotypic diversity of T cell lymphomas. Blood. 1986 Jun;67(6):1578–1582. [PubMed] [Google Scholar]
  16. Isaacson P. G., O'Connor N. T., Spencer J., Bevan D. H., Connolly C. E., Kirkham N., Pollock D. J., Wainscoat J. S., Stein H., Mason D. Y. Malignant histiocytosis of the intestine: a T-cell lymphoma. Lancet. 1985 Sep 28;2(8457):688–691. doi: 10.1016/s0140-6736(85)92930-7. [DOI] [PubMed] [Google Scholar]
  17. Isaacson P. G., Spencer J. Malignant lymphoma of mucosa-associated lymphoid tissue. Histopathology. 1987 May;11(5):445–462. doi: 10.1111/j.1365-2559.1987.tb02654.x. [DOI] [PubMed] [Google Scholar]
  18. Isaacson P. G., Spencer J., Wright D. H. Classifying primary gut lymphomas. Lancet. 1988 Nov 12;2(8620):1148–1149. doi: 10.1016/s0140-6736(88)90574-0. [DOI] [PubMed] [Google Scholar]
  19. Isaacson P. Malignant histiocytosis of the intestine: the early histological lesion. Gut. 1980 May;21(5):381–386. doi: 10.1136/gut.21.5.381. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Isaacson P., Wright D. H. Extranodal malignant lymphoma arising from mucosa-associated lymphoid tissue. Cancer. 1984 Jun 1;53(11):2515–2524. doi: 10.1002/1097-0142(19840601)53:11<2515::aid-cncr2820531125>3.0.co;2-c. [DOI] [PubMed] [Google Scholar]
  21. Isaacson P., Wright D. H. Intestinal lymphoma associated with malabsorption. Lancet. 1978 Jan 14;1(8055):67–70. doi: 10.1016/s0140-6736(78)90004-1. [DOI] [PubMed] [Google Scholar]
  22. Isaacson P., Wright D. H. Malignant histiocytosis of the intestine. Its relationship to malabsorption and ulcerative jejunitis. Hum Pathol. 1978 Nov;9(6):661–677. doi: 10.1016/s0046-8177(78)80049-5. [DOI] [PubMed] [Google Scholar]
  23. Jaffe E. S. Pathologic and clinical spectrum of post-thymic T-cell malignancies. Cancer Invest. 1984;2(5):413–426. doi: 10.3109/07357908409040316. [DOI] [PubMed] [Google Scholar]
  24. Jalkanen S. Lymphocyte homing into the gut. Springer Semin Immunopathol. 1990;12(2-3):153–164. doi: 10.1007/BF00197503. [DOI] [PubMed] [Google Scholar]
  25. Kanavaros P., Lavergne A., Galian A., Boivin P., Fourmestreaux A. R., Priollet B. C., Flandrin G., Hautefeuille P. A primary immunoblastic T malignant lymphoma of the small bowel, with azurophilic intracytoplasmic granules. A histologic, immunologic, and electron microscopy study. Am J Surg Pathol. 1988 Aug;12(8):641–647. [PubMed] [Google Scholar]
  26. Laszewski M. J., Kamat D., Kemp J. D., Goeken J. A., Mitros F. A., Platz C. E., Dick F. R. Immunophenotypic and genotypic characterization of primary non-Hodgkin's lymphoma of the gastrointestinal tract. Mod Pathol. 1990 Jul;3(4):423–428. [PubMed] [Google Scholar]
  27. Longacre T. A., Listrom M. B., Spigel J. H., Willman C. L., Dressler L., Clark D. Aggressive jejunal lymphoma of large granular lymphocytes. Immunohistochemical, ultrastructural, molecular, and DNA content analysis. Am J Clin Pathol. 1990 Jan;93(1):124–132. doi: 10.1093/ajcp/93.1.124. [DOI] [PubMed] [Google Scholar]
  28. Monoclonal antibody (HML-1) labelling of T-cell lymphomas. Lancet. 1989 Jan 28;1(8631):223–224. [PubMed] [Google Scholar]
  29. Mowat A. M. Human intraepithelial lymphocytes. Springer Semin Immunopathol. 1990;12(2-3):165–190. doi: 10.1007/BF00197504. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Musshoff K. Klinische Stadieneinteilung der Nicht-Hodgkin-Lymphome. Strahlentherapie. 1977 Apr;153(4):218–221. [PubMed] [Google Scholar]
  31. Möller P., Mielke B., Moldenhauer G. Monoclonal antibody HML-1, a marker for intraepithelial T cells and lymphomas derived thereof, also recognizes hairy cell leukemia and some B-cell lymphomas. Am J Pathol. 1990 Mar;136(3):509–512. [PMC free article] [PubMed] [Google Scholar]
  32. O'Farrelly C., Feighery C., O'Briain D. S., Stevens F., Connolly C. E., McCarthy C., Weir D. G. Humoral response to wheat protein in patients with coeliac disease and enteropathy associated T cell lymphoma. Br Med J (Clin Res Ed) 1986 Oct 11;293(6552):908–910. doi: 10.1136/bmj.293.6552.908. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Pals S. T., Meijer C. J., Radaszkiewicz T. Expression of the human peripheral lymph node homing receptor (LECAM-1) in nodal and gastrointestinal non-Hodgkin's lymphomas. Leukemia. 1991 Jul;5(7):628–631. [PubMed] [Google Scholar]
  34. Pinkus G. S., O'Hara C. J., Said J. W. Peripheral/post-thymic T-cell lymphomas: a spectrum of disease. Clinical, pathologic, and immunologic features of 78 cases. Cancer. 1990 Feb 15;65(4):971–998. doi: 10.1002/1097-0142(19900215)65:4<971::aid-cncr2820650425>3.0.co;2-b. [DOI] [PubMed] [Google Scholar]
  35. Radaszkiewicz T., Dragosics B., Bauer P. Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis. Gastroenterology. 1992 May;102(5):1628–1638. doi: 10.1016/0016-5085(92)91723-h. [DOI] [PubMed] [Google Scholar]
  36. Salter D. M., Krajewski A. S., Dewar A. E. Immunophenotype analysis of malignant histiocytosis of the intestine. J Clin Pathol. 1986 Jan;39(1):8–15. doi: 10.1136/jcp.39.1.8. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. Shepherd N. A., Blackshaw A. J., Hall P. A., Bostad L., Coates P. J., Lowe D. G., Levison D. A., Morson B. C., Stansfeld A. G. Malignant lymphoma with eosinophilia of the gastrointestinal tract. Histopathology. 1987 Feb;11(2):115–130. doi: 10.1111/j.1365-2559.1987.tb02616.x. [DOI] [PubMed] [Google Scholar]
  38. Spencer J., MacDonald T. T., Diss T. C., Walker-Smith J. A., Ciclitira P. J., Isaacson P. G. Changes in intraepithelial lymphocyte subpopulations in coeliac disease and enteropathy associated T cell lymphoma (malignant histiocytosis of the intestine). Gut. 1989 Mar;30(3):339–346. doi: 10.1136/gut.30.3.339. [DOI] [PMC free article] [PubMed] [Google Scholar]
  39. Sperling M., Kaudewitz P., Braun-Falco O., Stein H. Reactivity of T cells in mycosis fungoides exhibiting marked epidermotropism with the monoclonal antibody HML-1 that defines a membrane molecule on human mucosal lymphocytes. Am J Pathol. 1989 May;134(5):955–960. [PMC free article] [PubMed] [Google Scholar]
  40. Stansfeld A. G., Diebold J., Noel H., Kapanci Y., Rilke F., Kelényi G., Sundstrom C., Lennert K., van Unnik J. A., Mioduszewska O. Updated Kiel classification for lymphomas. Lancet. 1988 Feb 6;1(8580):292–293. doi: 10.1016/s0140-6736(88)90367-4. [DOI] [PubMed] [Google Scholar]
  41. Stein H., Dienemann D., Sperling M., Zeitz M., Riecken E. O. Identification of a T cell lymphoma category derived from intestinal-mucosa-associated T cells. Lancet. 1988 Nov 5;2(8619):1053–1054. doi: 10.1016/S0140-6736(88)90068-2. [DOI] [PMC free article] [PubMed] [Google Scholar]
  42. Stein H., Gerdes J., Schwab U., Lemke H., Mason D. Y., Ziegler A., Schienle W., Diehl V. Identification of Hodgkin and Sternberg-reed cells as a unique cell type derived from a newly-detected small-cell population. Int J Cancer. 1982 Oct 15;30(4):445–459. doi: 10.1002/ijc.2910300411. [DOI] [PubMed] [Google Scholar]
  43. Swinson C. M., Slavin G., Coles E. C., Booth C. C. Coeliac disease and malignancy. Lancet. 1983 Jan 15;1(8316):111–115. doi: 10.1016/s0140-6736(83)91754-3. [DOI] [PubMed] [Google Scholar]
  44. Tedder T. F., Isaacs C. M., Ernst T. J., Demetri G. D., Adler D. A., Disteche C. M. Isolation and chromosomal localization of cDNAs encoding a novel human lymphocyte cell surface molecule, LAM-1. Homology with the mouse lymphocyte homing receptor and other human adhesion proteins. J Exp Med. 1989 Jul 1;170(1):123–133. doi: 10.1084/jem.170.1.123. [DOI] [PMC free article] [PubMed] [Google Scholar]
  45. Tedder T. F., Penta A. C., Levine H. B., Freedman A. S. Expression of the human leukocyte adhesion molecule, LAM1. Identity with the TQ1 and Leu-8 differentiation antigens. J Immunol. 1990 Jan 15;144(2):532–540. [PubMed] [Google Scholar]
  46. Weis J. W., Winter M. W., Phyliky R. L., Banks P. M. Peripheral T-cell lymphomas: histologic, immunohistologic, and clinical characterization. Mayo Clin Proc. 1986 Jun;61(6):411–426. doi: 10.1016/s0025-6196(12)61975-2. [DOI] [PubMed] [Google Scholar]
  47. Weisenburger D. D., Linder J., Armitage J. O. Peripheral T-cell lymphoma: a clinicopathologic study of 42 cases. Hematol Oncol. 1987 Jul-Sep;5(3):175–187. doi: 10.1002/hon.2900050304. [DOI] [PubMed] [Google Scholar]
  48. Wolf B. C., Martin A. W., Ree H. J., Banks P. M., Smith S., Neiman R. S. Non-Hodgkin's lymphomas of the gastrointestinal tract. An evaluation of paraffin section immunostaining. Am J Clin Pathol. 1990 Feb;93(2):233–239. doi: 10.1093/ajcp/93.2.233. [DOI] [PubMed] [Google Scholar]

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