Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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Enteropathy-Type T-Cell Lymphoma Definition and Incidence Enteropathy associated T-Cell Lymphoma or enteropathy-type T-cell lymphoma (EATCL) is a tumour of intraepithelial T-lymphocytes showing varying degrees of transformation but usually presenting as a tumour composed of large lymphoid cells. This most commonly occurs in the setting of pre-existing or underlying (often undiagnosed) coeliac disease. These recommendations apply to EATCL but there is little evidence to suggest that non-EATCL should be treated differently. The median age of presentation is 50 years with a male predominance. ICD-O Code 9717/3 Clinical Presentation EATCL usually presents with abdominal pain, weight loss, diarrhoea and vomiting but may present acutely with small bowel obstruction and/or perforation. The tumour occurs most commonly in the jejenum or ileum with multiple ulcerating raised mucosal masses, one or more ulcers or a large exophytic mass. Pathology and Genetics The tumour cells are relatively monomorphic, medium sized to large cells with round or angulated vesicular nuclei, prominent nucleoli and moderate to abundant pale-staining cytoplasm. Less commonly, the tumour exhibits pleomorphism with multinucleated cells resembling anaplastic large cell lymphoma. Most tumours show infiltration by inflammatory cells, including large numbers of histiocytes and eosinophils. The adjacent intestinal mucosa usually shows features of enteropathy. Immunophenotype Tumour cells are CD3+, CD5-, CD7+, CD8+/-, CD4-, CD103+ and contain cytotoxic granule associated proteins. In most cases, a proportion of the tumour cells express CD30. The intraepithelial lymphocytes in the adjacent enteropathic mucosa may show an abnormal immunophenotype, usually CD3+, CD5-, CD8-, CD4-, identical to that of the lymphoma. Likewise the intraepithelial lymphocytes in refractory coeliac disease are usually CD8-. Genetics The TCR genes are clonally rearranged. Similar clonal rearrangements may be found in the adjacent enteropathic mucosa, suggesting that immunophenotypically aberrant intraepithelial lymphocytes are part of the neoplastic population. In refractory coeliac disease, the intraepithelial lymphocytes also comprise a monoclonal population and share the same clonal TCR gene rearrangements as the subsequent T-cell lymphomas which develops. Staging As for DLBCL. 47
Recommended Investigations Evaluation of patients with refractory coeliac disease Most patients with EATCL have a prior history of coeliac disease or simultaneous diagnosis of underlying coeliac disease. There is currently no recommendation for routine surveillance of coeliac disease patients who have responded to a gluten free diet. Refractory coeliac disease and ulcerative jejunitis probably represent a pre-neoplastic condition with frequent evolution to clonal disease and associated phenotypic changes. For these reasons a high level of suspicion in patients with coeliac disease with persistent symptoms is necessary. Immunophenotyping of the intraepithelial lymphocytes in serial biopsies in these patients may be useful in detecting phenotypic change (evolution to CD3+, CD8-, CD4- phenotype). Patients with established diagnosis of lymphoma Investigations should be carried out as for other high grade lymphomas. In addition, a small bowel follow through should be performed, and nutritional status assessed. Clonal T-cell populations may be identified in the peripheral blood, but the clinical significance of this is unclear. Treatment Standard treatment with anthracycline containing combination chemotherapy is usually used despite poor results. The Nottingham group have pioneered an approach using IEV chemotherapy (2 cycles) followed by 2 courses of Methotrexate 3gms/m2 for CNS prophylaxis followed by an autologous PBSCT with survival of 4 of 6 patients beyond 2 years. Gemctabine based therapy may be useful in aggressive T-NHLs such as EATCL. Perforation may occur during initial chemotherapy. Patients often require parenteral nutrition. In patients with refractory coeliac disease or ulcerative jejunitis, consideration may be given to pre-emptive treatment with combination chemotherapy if phenotypic change in the intraepithelial lymphocytes or clonally rearranged T-cells are detected, but this remains an experimental approach.. Response Evaluation and Follow Up Re-evaluation at intervals should include endoscopic evaluation, small bowel follow through and small bowel biopsy. Prognostic Factors / Index EATCL has a poor prognosis with a median survival of approximately 8 months and one year failure free survival of less than 20%. Potential Pitfalls Failure to recognise the development of EATCL in patients with refractory coeliac disease or ulcerative jejunitis. Frequent surveillance with endoscopic biopsy and immunophenotyping is recommended in patients with persistent or suspicious symptoms, and in those with refractory coeliac disease or with ulcerative jejunitis. 48
Page 1 and 2: Guidelines on Diag
Page 3 and 4: Guidelines on Diag
Page 5 and 6: Standards In Diagnosis of Lymphoma
Page 7 and 8: Standards in Staging of Lymphoma An
Page 9 and 10: Pathologic Diagnosis of Lymphoma Ge
Page 11 and 12: Hodgkin lymphoma and its differenti
Page 13 and 14: Potential diagnostic pitfalls for l
Page 15 and 16: Summary of the usual Immunostaining
Page 17 and 18: Mature B-Cell Neoplasms Mature B-Ce
Page 19 and 20: Immunophenotype The tumour cells ex
Page 21 and 22: Lymphoplasmacytic Lymphoma Definiti
Page 23 and 24: Splenic Marginal Zone Lymphoma Defi
Page 25 and 26: Extra-nodal Marginal Zone B-Cell Ly
Page 27 and 28: Persistent / progressive disease or
Page 29 and 30: Follicular Lymphoma Definition and
Page 31 and 32: Potential Pitfalls a. Failure to di
Page 33 and 34: Mantle Cell Lymphoma Definition and
Page 35 and 36: Diffuse Large B-Cell Lymphoma Defin
Page 37 and 38: Stage 1 (bulky) and stages II - IV
Page 39 and 40: Treatment Standard chemotherapy for
Page 41 and 42: Burkitt Lymphoma/Leukaemia Definiti
Page 43 and 44: CODOX-M, (3 cycles) for low risk di
Page 45 and 46: Potential pitfalls Failure to diffe
Page 47 and 48: Precursor T Cell Neoplasms PRECURSO
Page 49 and 50: Mature T-Cell and NK-Cell Neoplasms
Page 51: Potential Pitfalls Treatment planni
Page 55 and 56: Mycosis Fungoides and Sézary Syndr
Page 57 and 58: ■ ■ Lymph node biopsy if there
Page 59 and 60: Primary Cutaneous CD30-Positive T-C
Page 61 and 62: Angioimmunoblastic T-Cell Lymphoma
Page 63 and 64: Prognostic factors The IPI is of li
Page 65 and 66: Hodgkin Lymphoma (HL) Definition an
Page 67 and 68: Prognostic Factors / Index: i. Earl
Page 69 and 70: Immunodeficiency Associated Lymphop
Page 71 and 72: of cases and EBV in about 50%, but
Page 73 and 74: Common Issues in Lymphoma Managemen
Page 75 and 76: Tumour Lysis Syndrome Tumour Lysis
Page 77 and 78: Haematopoietic Recombinant Growth F
Page 79 and 80: Follow-Up Follow-up for patients wi
Page 81 and 82: Follow-up for patients with concern
Page 83 and 84: 77 Appendix
Page 85: Lower border: The lower of (i) 5 cm
Page 88 and 89: Glossary of Treatment Regimens Chlo
Page 90: 2nd Edition. May 2010. Supported by

Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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