Guidelines for the Management of Haematological Malignancies

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11 EXTRANODAL MARGINAL ZONE LYMPHOMA (MALT–type lymphoma) Key Issues • Identification of patients who require active treatment with chemotherapy • Great uncertainty about the impact of treatments on overall survival Diagnostic Criteria Typical Cases 1. Cellular composition includes small lymphocytes, centrocyte-like cells, 'monocytoid' B-cells, plasmacytoid cells. 2. Invasion of epithelial structures and existing germinal centres. 3. CD5 - , CD10 - , CD19 + , CD20 + , CD23 - , sIgM + , sIgD + or - . 4. Disease localized to or centred on an extranodal site. Assessment of residual disease The definitive diagnosis of residual disease requires the same criteria as at presentation. Solitary or multiple B-cell aggregates without germinal centres and cellular morphology suggestive of marginal zone lymphoma should be reported as suspicious and further follow up advised. The significance of molecular remission remains uncertain.(Bertoni, Conconi et al. 2002) Transformation The criteria are the same as for follicular lymphoma. The significance of increased numbers of large lymphoid cells is uncertain in otherwise typical cases. Prognostic Factors Extranodal marginal zone lymphoma with t(11;18)(Streubel, Simonitsch-Klupp et al. 2004) in the stomach may be resistant to helicobacter eradication. (Nomura, Yoshino et al. 2003) Essential Investigations • Bone aspirate and trephine • CT scan of thorax, abdomen and pelvis • Serum immunoglobulins • Direct Antiglobulin Test (DAT) • LDH 11.1 Gastric Marginal Zone Lymphoma Primary Treatment • Patients with Stage IE disease should receive Helicobacter pylori eradication as sole therapy. (Wotherspoon, Doglioni et al. 1993; Wotherspoon 1998) • Follow-up with upper GI endoscopy and biopsy every 6 months for the first 2 years. • Patients with recurrent disease after 1 year, stage greater than 1E or persistent and symptomatic disease with visible bulky disease after helicobacter eradication should be treated with chlorambucil (NCRI trial of chlorambucil vs. chlorambucil/ Rituximab IELSG19/MALT trial is closed now and is on follow up). (Level 1) • Patients with persistent / recurrent symptomatic disease despite chlormabucil may be treated with a purine analogue or single agent rituximab. Guidelines for the Management of Haematological Malignancies 11. EXTRANODAL MARGINAL ZONE LYMPHOMA 24
• Surgery is generally to be avoided except in emergency situations to control bleeding or repair a perforation; these are very rare in gastric marginal zone lymphoma • Radiotherapy is effective but usually not indicated given the excellent prognosis of this group of patients. • Patients with recurrent / persistent histological disease and macroscopically normal stomach do not require therapy Recurrence or resistance to above therapies • Patients with persistent/ recurrent asymptomatic disease after chlorambucil or trial therapy can be followed up by repeat endoscopy without further treatment. • For patients with large cell transformation, CHOP & Rituximab chemotherapy. (Level 1) Long-term follow-up(Zucca, Bertoni et al. 2000) • There is no need for upper GI endoscopy after resolution of lesions and eradication of H. pylori if patients are asymptomatic. • Endoscopy is only indicated if symptomatic. 11.2 Non-gastric extranodal MZL Primary Treatment(Zucca, Conconi et al. 2003) • Stage 1A disease may be treated with radiotherapy, if not in the trial, depending on the site of disease. • All other patients if asymptomatic – a watch and wait policy or enrolment in the NCRI extranodal lymphoma trial as mentioned above are reasonable options. • If treatment is required (e.g. > stage 1 disease) then patients should be considered for the NCRI extranodal lymphoma trial. • Chlorambucil is the usual 1st line therapy in non-trial patients. Relapse Treatment • If disease is disseminated, treat in a similar way to follicular NHL – single agent or combination chemotherapy as appropriate, eg. with chlorambucil or consider purine analogue or single agent rituximab • For patients with large cell transformation, CHOP & Rituximab chemotherapy. Guidelines for the Management of Haematological Malignancies 11. EXTRANODAL MARGINAL ZONE LYMPHOMA 25
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: • Splenectomy should only be cons
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
Page 71 and 72: 29 BINET DISEASE STAGE FOR CLL STAG
Page 73 and 74: 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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