Guidelines for the Management of Haematological Malignancies

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6 BURKITT LYMPHOMA Currently, the treatment strategy for this entity is also applied to high grade B cell NHL with a high proliferation rate (100% Ki67 +ve) as well as the more typical Burkitt Lymphoma – see diagnostic criteria below. Key issues • Effectiveness of short duration high intensity therapy remains unclear. (Mead, Sydes et al. 2002; Wang, Straus et al. 2003) • Continuing problems with diagnostic criteria. Diagnostic Criteria Burkitt lymphoma is defined as a germinal centre cell lymphoma with c-myc deregulation and absence of other balanced translocations. Typical Cases 1. Large B-cell lymphoma with round nuclei, central nucleoli and vacuolated cytoplasm. 2. Germinal centre phenotype: CD10 + , BCL-6 + by immunocytochemistry with BCL-2 - . 3. A hyperproliferative state demonstrated by Ki67 approaching 100%, p53 + , p21 - and evidence of apoptosis. 4. t(8;14) or variants demonstrated by FISH. Atypical Cases 1. Morphological variants - not clinically significant. 2. All above features except c-myc rearrangement - clinical significance not known. 3. All above features and t(14;18). Many of these patients have underlying follicular lymphoma. This is a poor prognostic feature. (Macpherson, Lesack et al. 1999) Essential Investigations • As for Diffuse Large B-cell lymphoma plus examination of the CNS in all cases. Primary Treatment • R-CODOX-M/R-IVAC study when available. • Treatment outside trial should be with R-CODOX-M/R-IVAC. • There are considerable issues regarding initial treatment toxicity and tumour lysis – Rasburicase pre-treatment should be considered especially in patients with high bulk and/ or abnormal renal function. • Patient should be treated in a level 2 centre or above. Relapsed disease There is no consensus or trial. Outcome would be expected to be very poor. There is little evidencebase for intensification of therapy and cases should be carefully reviewed in the relevant MDT. Guidelines for the Management of Haematological Malignancies 6. BURKITT LYMPHOMA 14
7 FOLLICULAR LYMPHOMA Key Issues • Role of high dose therapy in relapsed disease (Armitage 2001; van Besien, Loberiza et al. 2003)and poor risk patients. • Role of rituximab in primary and relapsed disease • Role of radiolabeled monoclonal antibodies • Role of reduced intensity allograft • Emergence of biological risk models • Concept of large cell transformation Diagnostic Criteria Follicular lymphoma is a tumour of the germinal centre cell that in lymph nodes shows a follicular growth pattern. Typical Cases In lymph node biopsy specimens the following features must be present: 1. The tumour must contain a mixture of cells with the morphology of centrocytes and centroblasts. 2. The tumour must have a germinal centre phenotype: • Immunocytochemistry: CD20 + , CD79 + , CD10 + , BCL-6 + , CD23 variable • Flow Cytometry: clonal sIgM or sIgG, CD19 + , CD10 + , CD38 + , CD5 - , and BCL-6 demonstrated by immunocytochemistry 3. BCL-2 expression by the neoplastic cells and/ or t(14;18) by FISH or PCR. 4. Partially or wholly follicular growth pattern. Variants 1. As above but BCL-2 and t(14;18) negative; clonality should be demonstrated by PCR or flow cytometry, or unequivocal evidence of bone marrow infiltration should be present. 2. Large cell variant where the neoplastic follicles consist mainly of centroblasts (WHO grade 3B). 3. Diffuse follicle centre lymphoma variant composed of a mixture of centrocytes and centroblasts with a germinal centre immunophenotype but lacking a follicular architecture. Transformation The diagnosis of transformation is made when diffuse areas are present that consist mainly of large lymphoid cells with a high rate of cell proliferation. The relationship between morphological transformation and progressive or refractory disease is complex. Essential Investigations • The International Prognostic Index modified for follicular lymphoma (FLIPI) should be calculated for all patients (Kondo, Ogura et al. 2001; Montoto, Lopez-Guillermo et al. 2002) • CT scan of thorax, abdomen, pelvis (and neck if clinically involved) • Bone marrow biopsy Primary Treatment Stage IA Although there is relatively little data there is some evidence that patients with stage 1A may be curable. All patients should be referred for consideration of radiotherapy. Guidelines for the Management of Haematological Malignancies 7. FOLLICULAR LYMPHOMA 15
Page 1 and 2: Yorkshire Cancer Network and Humber
Page 3 and 4: 18 MYELODYSPLASTIC SYNDROMES ......
Page 5 and 6: 2 IMAGING FOR HAEMATOLOGICAL MALIGN
Page 7 and 8: 3 CLASSICAL HODGKIN LYMPHOMA Key Is
Page 9 and 10: Assessments • Assessments should
Page 11 and 12: 5 DIFFUSE LARGE B-CELL LYMPHOMA (DL
Page 13: • Patients not considered candida
Page 17 and 18: 8 CHRONIC LYMPHOCYTIC LEUKAEMIA The
Page 19 and 20: Initial therapy for advanced CLL Th
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Page 23 and 24: • Splenectomy should only be cons
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Page 29 and 30: Relapse Treatment • Patients with
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Page 35 and 36: Guide to treatment options by stage
Page 37 and 38: 15.3 Plasmacytoma of Bone Diagnosti
Page 39 and 40: 15.6 Amyloidosis Primary and Relaps
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24.4 Acute Myeloid leukaemia There
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Data Collection The network is obli
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27 FLIP Index for Follicular Lympho
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29 BINET DISEASE STAGE FOR CLL STAG
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31 ECOG PERFORMANCE STATUS Grade EC
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33 WEBSITES UK Websites HMDS www.hm
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Dearden, C. E., E. Matutes, et al.
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Mead, G. M., M. R. Sydes, et al. (2
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35 Appendices DRAFT Haematological
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CT: Routine staging should include
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Multiple Myeloma and Related Disord
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Host factor, Microbiological and cl
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Guidelines for the Management of Haematological Malignancies

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