260 likes | 462 Views
A Beginner’s guide to Myeloma. Rod Johnson Leeds Teaching Hospitals. What are Plasma cells?. Differentiated B lymphocytes Antibody producing cells Part of our normal immune response. A Clonal Plasma Cell Disorder depends on the cells and the protein they make. MGUS Myeloma (symptomatic?)
E N D
A Beginner’s guide to Myeloma Rod Johnson Leeds Teaching Hospitals
What are Plasma cells? • Differentiated B lymphocytes • Antibody producing cells • Part of our normal immune response
A Clonal Plasma Cell Disorderdepends on the cells and the protein they make • MGUS • Myeloma (symptomatic?) • Plasmacytomas • Bony or soft tissue • solitary/multiple • Other paraprotein disorders • Amyloid, neuropathies etc
MGUSMonoclonal Gammopathy of UndeterminedSignificance (!!) • Common, 3-5% of the elderly • Only a third become Myeloma • Some are other diseases (e.g. amyloid) • Can we predict progression? • How often to monitor…..
Epidemiology of Myeloma • 1% of all malignancy (2% Afro-Caribbean) • Annual UK incidence about 6/100,000 • Male:Female ratio is 1.6:1 • Median age at diagnosis is 70 • 10-15% diagnosed <50 yrs • 5% diagnosed <40 yrs
Presenting Features • Bone pain, commonly lumbar spine • Anaemia, often slightly macrocytic • Recurrent infections , mainly chest • Hypercalcaemia • Renal failure • Hyperviscosity (paraprotein) • Amyloid
Diagnosis • Serum and/or urine Paraprotein • Typical bone changes • Marrow excess of plasma cells
Principles of Treatment • Not really a curable disease • Plasmacytomas / ‘operational cures..’ • Treatment dose extend survival • Disease activity correlates with symptoms • Median survival is 48 months
Treatment Options 1.Supportive Care: • Pain control • Radiotherapy or surgery • Control of calcium / Bisphosphonates • Renal support (fluids, ?dialysis) • Blood product support • Management of infection
Treatment options 2.Conventional chemotherapy • Melphalan / Cyclophosphamide • Steroids alone or in addition • Combination Chemo. (ABCM)
Treatment options 3.High dose chemotherapy Infusional chemotherapy High dose melphalan Stem Cell Transplant
Transplant Strategies Autograft Allograft 'Mini' Allograft Total Therapy Double autograft
Newer Agents(if you can afford them…..) • Thalidomide • Other iMIDS (Lenalidomide) • Velcade (Bortezomib) • Future biological agents
Thalidomide • Now established therapy • Has its own particular side effects: • Drowsiness • Constipation • Neuropathy • Thrombosis • Used in various combinations • ?Maintenance therapy…..
Difficult Situations • The elderly patient • The young patient • (The ones in between !!) • The relapsed patient • Renal Failure • Bony/neurological damage • Amyloid
Amyloid • Also responds to Myeloma therapy • Dissolve the protein…………?
Conclusions 1.(The specialist context) • Fascinating new insights into this disease • A wealth of new agents becoming available • Overall survival has improved • Better treatment for older age groups • It costs a lot!
Conclusions 2.(The real context) • Much renewed interest in this disease • A Disease of the elderly • Unpleasant and debilitating • Remains largely incurable • A major problem for continuing care