Guidelines for the Management of Haematological Malignancies

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explains the resistance to conventional chemotherapeutic agents. The only licensed therapy for fludarabine-refractory CLL is alemtuzumab. The response rates to alemtuzumab are most influenced by the size of lymphadenopathy. The duration of remission and survival following alemtuzumab are related to the depth of remission including whether minimal residual disease is eradicated. It is recommended that these patients are referred for consideration by the specialist CLL MDT. The following approaches are indicated for patients with refractory CLL who are considered suitable for intensive treatment: CLL206(CamPred) trial: Phase II trial for Fludarabine refractory patients with p53 deletion analysing the efficacy of high dose steroids and alemtuzumab. (Level 1 or 2) Patients with predominantly blood and marrow disease with relatively small lymph nodes should be treated with alemtuzumab. (Moreton, Kennedy et al. 2005)The aim of therapy should be to eradicate detectable minimal residual disease from the marrow. Patients with bulky lymphadenopathy should receive initial therapy to reduce the bulk of lymph nodes prior to treatment with alemtuzumab in order to eradicate detectable minimal residual disease. Highdose methyl prednisolone (1g/m 2 /day for 5 days every 4 weeks) is effective in patients for whom high dose steroids are not contra-indicated. (Level 1) Allogeneic stem cell transplantation CLL is an incurable disease by conventional treatment approaches. The transplant related morbidity and mortality for allogeneic stem cell transplantation is very high. However there is a significant graftversus-CLL effect and long-term disease free survival is achievable following allogeneic stem cell transplantation. Patients who are possible candidates for allogeneic stem cell transplantation should be considered by the MDT in Leeds on a case-by-case basis. Patients are generally only considered for allogeneic transplantation if they have poor-risk disease either according to biological characteristics of their CLL or because they have fludarabine-refractory disease. (Level 4) Guidelines for the Management of Haematological Malignancies 8. CHRONIC LYMPHOCYTIC LEUKAEMIA 20
9 MANTLE CELL LYMPHOMA Key Issues Uncertainty as to the efficacy of current therapy (Densmore and Williams 2003; Hiddemann and Dreyling 2003) Diagnostic Criteria Typical Cases 1. Monomorphic population of small to intermediate sized B-cells 2. Phenotype: sIg ++/+++ (IgM & IgD), CD5 + , CD23 - , CD20 + 3. BCL-1 expression/ t(11;14) Variants Blastic or large cell variant associated with an aggressive clinical course Essential Investigations • Bone marrow aspirate and trephine • CT scan of thorax, abdomen and pelvis (and neck if clinically involved) • Calculation of International Prognostic Index • Gastrointestinal investigations e.g. Upper GI endoscopy/ sigmoidoscopy and biopsy, if symptomatic • Consideration of possible allogeneic transplant at time of presentation Primary Treatment • A Phase 3 randomised controlled trial of Fludarabine/ cyclophosphamide combination with or without Rituximab in patients with untreated MZL is available and all eligible patients should be treated under trial if possible. (Level 1) • Patients who are not in trial: Young and fit patients -should receive FC (Fludarabine and cyclophosphamide), FC Rituximab, HyperCVAD-R, or CHOP-R and considered for Autotransplant (Level 1,2 or 3 decided by MDT) Older patients- if asymptomatic and nonprogressive disease should be observed. Patients who are unfit for more aggressive treatments -should be considered for chlorambucil. Relapse Treatment • Patients who didn’t have an Autograft in the primary treatment should be considered for Autograft.(Level 3) • Alternate front line regimen should be used for those who can’t have a transplant. • Young fit patients who achieve a response should be considered for an allogeneic stem cell transplant (conventional conditioning or reduced intensity conditioning). (Level 4) • Thalidomide, especially combined with rituximab has a potential action but there is poor data regarding this. (Level 1) Guidelines for the Management of Haematological Malignancies 9. MANTLE CELL LYMPHOMA 21
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 and 14: • Patients not considered candida
Page 15 and 16: 7 FOLLICULAR LYMPHOMA Key Issues
Page 17 and 18: 8 CHRONIC LYMPHOCYTIC LEUKAEMIA The
Page 19: Initial therapy for advanced CLL Th
Page 23 and 24: • Splenectomy should only be cons
Page 25 and 26: • Surgery is generally to be avoi
Page 27 and 28: 13 PERIPHERAL T-CELL LYMPHOMA Key I
Page 29 and 30: Relapse Treatment • Patients with
Page 31 and 32: Essential Investigations • CT sca
Page 33 and 34: • Peripheral blood for flow cytom
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
Page 41 and 42: Primary Treatment BCSH guidelines s
Page 43 and 44: Patients aged 15-24 (up to 25th bir
Page 45 and 46: 18 MYELODYSPLASTIC SYNDROMES Diagno
Page 47 and 48: • Both the degree of thrombocytop
Page 49 and 50: • Patients presenting in blast cr
Page 51 and 52: A more difficult decision concerns
Page 53 and 54: Diagnostic Criteria With the except
Page 55 and 56: 21.4 Reporting Standards All specim
Page 57 and 58: All members of Haemato-oncology MDT
Page 59 and 60: 22.3 Clinical Support • On-site a
Page 61 and 62: • Principle treatment centres mus
Page 63 and 64: • It is envisaged that relevant s
Page 65 and 66: 24.4 Acute Myeloid leukaemia There
Page 67 and 68: Data Collection The network is obli
Page 69 and 70: 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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