Guidelines for the Management of Haematological Malignancies

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19 CHRONIC MYELOID LEUKAEMIA Diagnostic Criteria Chronic phase CML • t(9;22) as part of a single or complex translocation, or BCR-ABL transcript must be present in every case • Maximally cellular marrow with left shifted, myeloid series, monolobated megakaryocytes and suppressed erythroid series • 20% basophils in marrow or blood, associated with clinical progression or refractory to treatment. • Blast crisis (BC): >20% myeloid blasts or >5% lymphoid blasts or solid extramedullary tumour deposit consisting mainly of blasts. Essential Investigations • Full blood count with manual differential. • Full examination with documentation of liver and spleen size. Ultrasound scanning of the abdomen to more accurately document spleen size may be considered. • Routine biochemistry to include U&Es, LFTs, calcium, LDH and urate. • Bone marrow aspirate and trephine with sample sent for cytogenetics • Bone marrow and peripheral blood sample for RT-PCR • All newly diagnosed patients should have a Hasford or New CML (Euro) score measured. This can be calculated using a simple web based programme found at www.pharmacoepi.de. • A full family history, in particular paying attention to potential sibling donor details, should be taken. As early as possible after diagnosis, tissue typing of the patient and siblings should be arranged. This should be mandatory in all patients under the age of 65. Primary Therapy (Oscier, Fegan et al. 2004) Patients in Chronic Phase (CP) • All new patients should be offered entry into the SPIRIT study (Level1) • All patients are to be considered for stem cell harvest. However, in practice this should only be carried out in patients who may be eligible for studies assessing the role of autografting in CML, or in whom a backup for allogeneic transplantation may be required. • In patients with symptoms of leucostasis consideration may be given to therapeutic leucopheresis in addition to buffy coat stem cell collection. • All patients should be well hydrated and receive allopurinol 300-600 mg daily. • Initial cytoreductive therapy should be with Imatinib (400 mg daily). (Level 1) Rarely, patients may be encountered who are intolerant of Imatinib. Treatment options in such cases should be discussed with the MDT lead for leukaemia,as new trials will become available in the next near future investigating new targeted therapies. Patients in Accelerated Phase (AP) • Patients presenting in the accelerated phase of CML should receive Imatinib 600mg daily, which is associated with a reported progression-free survival rate of 67% at 12 months, and overall survival of 66% at 36 months. Patients in Blast Crisis (BC) Guidelines for the Management of Haematological Malignancies 19. CHRONIC MYELOID LEUKAEMIA 48
• Patients presenting in blast crisis present a difficult management problem. The best responses have been reported to Imatinib 600mg daily, with reported 12 month survival rates of 32%. Major cytogenetic responses have been reported in 16% of patients, and complete cytogenetic responses in 7%. NICE do not recommend continued use of Imatinib in patients presenting in CP who progress to AP or BC after exposure to the drug. Management of such cases should be discussed with the MDT lead for leukaemia. For patients in BC, options include acute leukaemia type therapy (as for AML or ALL depending on phenotype) or autologous stem cell transplantation. Patients in AP and BC mayalso be eligible for new trials of alternative targeted therapies(see above). Further Management of Chronic Myeloid Leukaemia Patients on Imatinib Imatinib is well tolerated by the majority of patients. • Side effects include mild allergic type rashes, which may respond to simple anti-histamines. • Severe dermatological reactions have been documented which may require steroid therapy or in some cases the discontinuation of Imatinib. • Arthralgia is seen in 60% of patients and usually responds to simple treatment with nonsteroidals. • Fluid retention may be troublesome and occurs in the majority of cases often just as simple peri-orbital oedema. More severe oedema is often quite refractory to diuretic therapy and may require dose reduction or discontinuation. • Neutropenia is usually easily managed by the addition of GCSF to the regimen and may allow dose escalation. • Patients in whom there is no documented haematological or cytogenetic response may benefit from dose escalation of up to 800 mg per day. Again the addition of GCSF may be helpful in maintaining dose levels. • The combination with α-interferon has been tried and has produced cytogenetic responses in patients not achieving this with α-interferon alone. The most experience has been with once weekly PEG-Intron in doses up to 1µg/kg/week in the PISCES study. Management of Chronic Myeloid Leukaemia Patients who are resistant or intolerant to Imatinib • In chronic phase for treatment failure new tyrosine kinase inhibitors like Nilotinib or Dasatinib(Talpaz, Shah et al. 2006) should be tried before considering Allogenic stem cell transplant if appropriate. • For suboptimal response the options are either dose escalation of Imatinib to 600-800mg or changing to new tyrosine kinase inhibitors should be considered before offering Allogenic stem cell transplant.(Hughes, Deininger et al. 2006) • For accelerated phase and blast crisis new tyrosine kinase inhibitors and Allogenic stem cell transplant are the options. Monitoring of Response to Imatinib(Goldman 2005; Baccarani, Saglio et al. 2006) • All patients should have baseline bone marrow examination with cytogenetic analysis and peripheral blood sample for quantitative RT-PCR. • Peripheral blood monitoring should be done every 3 months. Bone marrow investigation will be advised for patients with inadequate response. 10ml of EDTA blood is required. At 3 months: If 1 log reduction not achieved (>5.5%) then that is unsatisfactory response. Bone marrow cytogenetics and peripheral blood PCR at 6 months. If 2 log reduction is achieved (0.55% or less), this is probably equivalent to a complete cytogenetic response. Then continue 3 monthly PB assessment. At 12 months of therapy If at least 3 log reduction (>0.055% or less) is achieved then it is considered as a major molecular response bone marrow cytogenetics should be performed with peripheral blood Guidelines for the Management of Haematological Malignancies 19. CHRONIC MYELOID LEUKAEMIA 49
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 and 20: Initial therapy for advanced CLL Th
Page 21 and 22: 9 MANTLE CELL LYMPHOMA Key Issues U
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: • Both the degree of thrombocytop
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
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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
Page 71 and 72: 29 BINET DISEASE STAGE FOR CLL STAG
Page 73 and 74: 31 ECOG PERFORMANCE STATUS Grade EC
Page 75 and 76: 33 WEBSITES UK Websites HMDS www.hm
Page 77 and 78: Dearden, C. E., E. Matutes, et al.
Page 79 and 80: Mead, G. M., M. R. Sydes, et al. (2
Page 81 and 82: 35 Appendices DRAFT Haematological
Page 83 and 84: CT: Routine staging should include
Page 85 and 86: Multiple Myeloma and Related Disord
Page 87: Host factor, Microbiological and cl

Guidelines for the Management of Haematological Malignancies

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