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34 - Management of the lymphomas and myeloma
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- By Eve Gallop-Evans, Velindre Cancer Centre, Velindre Hospital, Cardiff, UK
- Edited by Louise Hanna, Tom Crosby, Fergus Macbeth
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- Book:
- Practical Clinical Oncology
- Published online:
- 05 November 2015
- Print publication:
- 19 November 2015, pp 450-472
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Summary
Introduction
The incidence of lymphoma is rising, and current attention is focused not just on improving cure rates, but also on minimising late effects of treatment. Patients should be managed by multidisciplinary teams that bring together the appropriate expertise of haematologists, oncologists, radiologists, pathologists and specialist nurses. Management guidelines are produced by the British Committee for Standards in Haematology (BCSH) and are a useful resource.
Lymphomas
Introduction
The World Health Organisation (WHO) classification of neoplasms of the hematopoietic and lymphoid tissues was first published in 2001 and updated in 2008 (Table 34.1: Swerdlow et al., 2008). This distinguishes more than 60 specific entities on the basis of morphologic, immunophenotypic, genetic, molecular and clinical features, stratified according to cell lineage and derivation from precursor or mature lymphoid cells.
Non-Hodgkin lymphoma (NHL) is the sixth most common cancer in the UK, with over 12,000 patients diagnosed each year, accounting for 4% of all new cases of cancer. The incidence is related to age, with the majority of cases diagnosed over the age of 65 years. Nearly half of all cases diagnosed in the UK are diffuse large B cell lymphoma (DLBCL, 48%). Marginal zone lymphomas (MZL) and follicular lymphoma (FL) account for 20% and 19%, respectively, with T cell lymphomas (6%), mantle cell lymphoma (MCL, 5%) and Burkitt lymphoma (2%) making up the remainder. Crude incidence rates in the UK range from 0.2 to 9 cases per 100,000.
Hodgkin lymphoma (HL) accounts for 0.6% of all cancer cases, with a relatively stable incidence of around 1700 cases per year. In children under 14 years of age, lymphoma is the third most common cancer after leukaemia and brain tumours. Lymphomas are the most common cancer in teenagers and young adults, accounting for 21% of cancers in this age group, with two-thirds of these being HL.
Aetiology
The aetiology of lymphoma is not clearly understood and appears to be multifactorial. The most significant risk factor is immune dysfunction which may be secondary to viral infection (e.g. HIV, HBV, HCV, EBV, HTLV-1), autoimmune disease or iatrogenic immunosuppression (Roman and Smith, 2011). Mucosa-associated lymphoid tissue (MALT) lymphomas are associated with antigenic stimulation by infectious agents including Helicobacter pylori, Chlamydia psittaci and Borrelia burgdorferi.
13 - Solitary bone and extra-medullary plasmacytoma
- from Section 3 - Myeloma: clinical entities
- Edited by Stephen A. Schey, Kwee L. Yong, Robert Marcus, Kenneth C. Anderson
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- Book:
- Myeloma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 167-173
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Summary
Introduction and epidemiology
Solitary plasmacytoma, which accounts for less than 5% of plasma cell dyscrasias is characterized by a localized proliferation of malignant plasma cells in the absence of evident disease elsewhere. Such a proliferation may arise in a bone (solitary bone plasmacytoma or SBP) or an extra-medullary compartment (extramedullary plasmacytoma or EMP). Plasmacytomas may also arise in a multifocal manner without evidence of malignant plasma cells in the intervening tissues. Extra-medullary plasmacytomas may also arise in the context of multiple myeloma.
Solitary Bone Plasmacytoma
SBP may arise at any age, but the median age of onset at 55 years is 10 years younger than that for myeloma, with a 1.87:1 male to female ratio[1].
Clinical and laboratory features
In two-thirds of cases, SBP arises in the axial skeleton, including the spine (thoracic > lumbar > sacral > cervical spine), skull, ribs and sternum and, in one-third, the appendicular skeleton, including the shoulder girdle, pelvic girdle or the extremities [2]. Localized skeletal pain due to the solitary osteolytic lesion is a typical presentation of SBP. If the spine is affected, spinal cord or nerve root compression may be an important clinical consequence. Other presentations include pathological fracture of the affected bone, a soft tissue mass due to extra-medullary extension of the tumor and in a small proportion, symptoms of peripheral neuropathy.
31 - The lymphomas and myeloma
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- By Eve Gallop-Evans, Consultant, Clinical Oncologist, Velindre Cancer Centre, Velindre Hospital, Whitchurch, Cardiff, UK, Chris Poynton, Senior Lecturer and Honorary Consultant, Haematologist, University Hospital of Wales, Heath Park, Cardiff, UK
- Edited by Louise Hanna, Tom Crosby, Fergus Macbeth
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- Book:
- Practical Clinical Oncology
- Published online:
- 23 December 2009
- Print publication:
- 24 January 2008, pp 347-369
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Summary
Introduction
The haematological malignancies make up a group of diverse diseases ranging from the very indolent, which may co-exist with the patient for many years, to the highly aggressive and rapidly fatal. They can occur anywhere in the body, and so this chapter is based on tumour type rather than anatomical site, although a detailed anatomical knowledge is required for treatment planning.
Apart from making a tissue diagnosis, surgery rarely has a part in the management of these patients, thus, the development of curative treatment protocols for many of these patients has been a success story for chemotherapy and radiotherapy (RT). With more patients surviving longer, attention is focussing on minimising the unwanted late effects of treatment as much as on maximising the chances of a cure. Patients should be managed by multidisciplinary teams that bring together the appropriate expertise of haematologists, oncologists, radiologists, pathologists and specialist nurses.
This chapter first considers the lymphomas, namely non-Hodgkin lymphoma and Hodgkin lymphoma. Then it considers the paraproteinaemias, which include multiple myeloma and solitary plasmacytoma. Lastly, it considers total-body irradiation, which is given as preconditioning prior to bone marrow transplantation in some lymphomas and leukaemias. Leukaemia in children is considered in Chapter 37 (see p. 431). However, a detailed discussion of adult leukaemias is outside the remit of this book.
Lymphomas: general aspects
Introduction
Non-Hodgkin lymphoma (NHL) is the seventh most common cancer in the UK, with a yearly incidence of about 9000 new cases, and occurs mostly in patients over age 65.