Guidelines on Diagnosis and Treatment of Malignant Lymphomas

More documents

Recommendations

Info

Cutaneous ALCL (in contrast to systemic ALCL) express cutaneous lymphocyte antigen but not EMA, ALK and rarely CD15. CD56 expression is rare and has no prognostic significance. Genetics The T-cell receptor genes are clonally rearranged in most cases. Cutaneous ALCL does not have ALK associated translocations. Staging Disease is usually confined to the skin at diagnosis. Patients with primary cutaneous ALCL should be staged as for DLBCL. Recommended Investigations As for DLBCL. Potential Pitfall(s) Failure to distinguish primary skin lymphoma from systemic Anaplastic Large Cell Lymphoma with cutaneous involvement and from secondary high grade lymphomas with CD30 expression. Response Evaluation and Follow Up Clinical skin examination for skin disease,. Prognosis Both conditions have an excellent prognosis with 100% and 96% 5-year survival for LyP and cutaneous ALCL. Patients with multifocal skin lesions and those with involvement of regional lymph nodes have a similar prognosis to patients with skin lesions only. Treatment Patients with LyP may not require treatment. In patients with either LyP or primary cutaneous CD30+ ALCL requiring therapy, PUVA, local radiotherapy or low-dose oral methotrexate are effective at preventing recurrent lesions. Systemic chemotherapy is usually not effective.. 55
Angioimmunoblastic T-Cell Lymphoma Definition and Incidence Angioimmunoblastic T-Cell Lymphoma, AILT (formerly AILD) is a peripheral T-cell lymphoma characterised by systemic symptoms, a polymorphous infiltrate involving lymph nodes, with a prominent proliferation of high endothelial venules and follicular dendritic cells. It occurs in the middle aged and elderly, with an equal incidence in males and females. ICD-O Code 9705/3 Clinical Presentation Patients usually present with B symptoms, generalised peripheral lymphadenopathy, hepatosplenomegaly, and frequent skin rash. The bone marrow is commonly involved. Para-neoplastic manifestations are common and include skin rashes, autoimmune haemolytic anaemia, hypergammaglobulinaemia, eosinophilia, vasculitis, and haemophagocytosis. The clinical course is aggressive, with a median survival of less than 3 years. Pathology and Genetics The lymph node architecture is partially effaced, and regressed follicles are often present. The paracortex is diffusely infiltrated by a polymorphous population of medium-sized lymphocytes, usually with clear to pale cytoplasm and distinct cell membranes. The lymphocytes show minimal cytological atypia, and this form of lymphoma may be difficult to distinguish from atypical T-zone hyperplasia. The abnormal lymphoid cells are admixed with small, reactive lymphocytes, eosinophils, plasma cells and histiocytes. There is marked proliferation of high endothelial venules and follicular dendritic cell meshworks are often increased. Increased numbers of B immunoblasts are usually present in the paracortex. Immunophenotype The infiltrates are composed of mature T-cells, usually with an admixture of CD4 and CD8 cells, with CD4 cells usually outnumbering CD8 cells. Follicular dendritic cells (CD21+) are prominent. The neoplastic T-cells may aberrantly express CD10. Genetics T-cell receptor genes are rearranged in 75% of cases. Immunoglobulin gene rearrangement is present in 20-30%, correlating with clonally expanded EBV+ B cells. Gene expression studies confirm that the neoplastic cells are CD4+ TFH type. Staging As for other aggressive lymphomas. Recommended Investigations As for other aggressive lymphomas. Potential Pitfalls Failure to diagnose lymphoma in the context of a clinical presentation characterised by a profound immune disturbance. Treatment Treatment options include CHOP or CVP-like regimens, depending on patient age and co-morbidity. There is a high initial response rate with these approaches, but early relapse or progression is common and there are few long-term survivors. Long term disease control may be achieved with weekly methotrexate and prednisolone. High dose therapy and autologous SCT have been reported by the EBMT to results in a 5 year 60% disease free survival. Response Evaluation and Follow Up As for other aggressive lymphomas. 56
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 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: Primary Cutaneous CD30-Positive T-C
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
show all

Guidelines on Diagnosis and Treatment of Malignant Lymphomas

You also want an ePaper? Increase the reach of your titles

Delete template?

Save as template?