Guidelines on Diagnosis and Treatment of Malignant Lymphomas

More documents

Recommendations

Info

Introduction In Ireland and Northern Ireland approximately 800 new cases of malignant lymphoma, Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), are registered each year. In line with the rest of the western world, the incidence of NHLs appears to be increasing, probably by 3-5% per year and, being largely a disease of the elderly, these tumours represent an increasing cause of cancer-related death. The reasons for increasing incidence are multiple, ranging from the increasing elderly population, greater accuracy of diagnosis and unknown environmental factors. The increase has not been noted in HL and in all subtypes of NHL and cannot solely be explained on the basis of the HIV epidemic. NHLs are a heterogeneous group of diseases which are mainly linked by their origin within the lymphoid system and its different cellular components. Over 60% of NHLs are accounted for by two diseases, diffuse large B-cell lymphoma (DLBCL) which is an aggressive disease and follicular lymphoma (FL) which is an indolent or non-aggressive entity. Many other less- common and often rare sub-types of NHL exist. All display distinct natural histories and require complex diagnostic approaches and management. The Lymphoma Forum of Ireland (LFI) first met in October 2002 and outlined its aims as: the promotion of a high standard of care and the development of all aspects of lymphoma diagnosis and treatment in Ireland and Northern Ireland, development and fostering of research initiatives relating to malignant lymphomas and interaction with other relevant bodies towards achieving these aims. Members of the LFI include medical oncologists, haematologists, pathologists and radiation oncologists involved in the diagnosis and management of malignant lymphomas. After a number of exploratory meetings, it was agreed that the initial step required was the development of management guidelines for these diseases in line with the WHO classification and best international treatment practices. As an initial effort, guidelines for the diagnosis and management of 22 diseases have been developed as well as guidelines for the management of some generic issues. This is the first update of the guidelines issued in parallel with the updated WHO guidelines published in 2008 and these will be made available on the Haematology Association of Ireland website. Over the last forty years there have been numerous efforts to classify lymphoid malignancies culminating in the WHO classification introduced after much international effort in 2001. It is clear that malignant lymphomas require a sophisticated diagnostic approach based on clinical features, morphology, immunophenotyping and genetic analysis. It is essential that such an approach underpins the clinical management of these diseases, many of which are amenable to cure. Clinical care should be delivered by those with expertise and experience in the area, within a multidisciplinary setting. 1
Standards In Diagnosis of Lymphoma Tissue collection Investigations prior to biopsy A full blood count (FBC) and film (with flow cytometry if appropriate), should be carried out before a node biopsy to avoid biopsying patients with CLL or acute leukaemia. Monospot: in patients < 30 years with lymphadenopathy. Epithelial carcinoma should be considered in patients >40 with head and neck adenopathy, who should have an ENT examination and FNA. Designated surgeons should perform all lymph node biopsies in lymphoma diagnosis A designated surgeon ensures appropriate and uniform specimen collection and prompt referral of patients to the lymphoma service. The preliminary biopsy report should be available to the multidisciplinary team (MDT) within 2 weeks of the patient’s hospital referral. An excision lymph node biopsy is preferable for diagnosis An excision biopsy allows detailed assessment of architecture, which is a key feature in lymphoma diagnosis. Needle biopsies are more prone to artefact and may be inadequate for all the diagnostic investigations. A lymph node biopsy is preferable to a biopsy of an extra-nodal site. Approach to diagnosis of a patient with lymphadenopathy ■ FBC with film (and cell marker studies where appropriate) ■ Monospot in patients < 30 years ■ Consider ENT examination and FNA to exclude epithelial malignancy of the head and neck in patients >40 ■ Designated surgeon(s) ■ Excision biopsy preferred method; trucut biopsy if node not accessible ■ Node biopsy – send unfixed to laboratory Lymph node biopsies should be sent fresh to the laboratory This requires local arrangements for the prompt and safe transport of the specimen. Fresh material is essential for good quality histology and facilitates the use of new diagnostic techniques. See Royal College of Pathologists minimum dataset for lymphoma reports. Laboratory diagnosis Sample handling In the laboratory, the lymph node should be sliced and imprint preparations made. Thin slices should be placed in formalin for 24 hours before processing as paraffin blocks. This is essential for high-quality morphology and reproducible results with marker studies performed on paraffin sections. The remaining tissue may be snap frozen and disaggregated into a single-cell suspension. 2
Page 1 and 2: Guidelines on Diag
Page 3: Guidelines on Diag
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 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
show all

Guidelines on Diagnosis and Treatment of Malignant Lymphomas

Create successful ePaper yourself

Delete template?

Save as template?