Guidelines on Diagnosis and Treatment of Malignant Lymphomas

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B Cell Lymphoma Definition and Incidence Aggressive large cell B cell lymphomas with morphologic and genetic features of both DLBCL and Burkitt lymphoma but with atypical features which preclude classification with either of these entities. Some of these cases were previously classified as Burkitt-like lymphoma. Some cases have mixed morphology intermediate between DLBCL and BL, others have typical morphology of BL but atypical immunophenotype or genetic features. This disease category is heterogenous, infrequently diagnosed, and is not a distinct entity but allows classification of cases which are impossible to classify as classical DLBCL or BL. ICD – O Code: 9680/3 Clinical Presentation Many cases present with extra-nodal disease but there is no particular association with ileocaecal or jaw location. Bone marrow and peripheral blood may be involved. Pathology There is a diffuse proliferation of medium to large sized lymphoid cells with frequent mitotic and apoptotic activity and many macrophages with a “starry sky” appearance. Nuclear morphology is more variable than in classical BL including variation in nuclear size, nuclear irregularity and/or prominent nucleoli. In some cases morphology is typical of BL but immunophenotype and/or genetic features are not. Occasional cases have smaller nuclei resembling lymphoblasts. Cases of morphologically typical DLBCL with very high proliferation fraction should NOT be included. Immunophenotype B cell markers are positive, including CD20, CD19 and CD79a. Surface Ig is typically positive. Ki67 labelling index is usually very high. Many cases in this category demonstrate a typical immunophenotype for BL (CD10+, BCL6+, BCL2- IRF4/MUM1-) but atypical morphology. Cases with typical morphology of BL but atypical immunophenotype (BCL2+) are also included although such cases may also represent a “double-hit” phenomenon with both BCL2 and MYC translocations. Genetics Clonal Ig gene rearrangements are present. 35-50% of cases have 8q24/MYC translocation but unlike BL many of these are non IG-MYC translocations. BCL2 translocation is present in up to 15% of cases and may be associated with MYC (“double hit”) and/or BLC6 translocations. A complex karyotype is frequent, unlike BL. Staging As for Burkitt Lymphoma Recommended investigations As for Burkitt Lymphoma 41
Potential pitfalls Failure to differentiate from DLBCL Treatment Standard DLBCL therapy is unlikely to be curative. Chemotherapy regimens used in Burkitt’s lymphoma are active. Another approach is to use DA-R-EPOCH, which is more anthracycline intensive than classical Burkitt’s regimens. Patients should receive CNS directed therapy. Patients with double hit lymphomas originating from a follicular lymphoma have a very poor prognosis. Response evaluation and Follow-up Restage after first course of chemotherapy (Burkitt regimen) or second / fourth course with DA-R-EPOCH and then at the end of treatment. The risk of relapse remains high for up to 2 years following treatment compared to Burkitt’s lymphoma 42
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: CODOX-M, (3 cycles) for low risk di
Page 47 and 48: Precursor T Cell Neoplasms PRECURSO
Page 49 and 50: Mature T-Cell and NK-Cell Neoplasms
Page 51 and 52: Potential Pitfalls Treatment planni
Page 53 and 54: Recommended Investigations Evaluati
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

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