ABSTRACT

Plasma cell myeloma 1299 Introduction 1299 Clinical features 1300 Diagnostic criteria 1302 Prognostic indicators 1302 Management 1303 Response criteria 1303 Induction therapy 1305 High-dose therapy 1305 New agents 1308 Supportive care 1309 Follow-up 1309 Monoclonal gammopathy of undetermined significance 1310 Introduction 1310 Diagnostic criteria 1310 Epidemiology and risk factors 1310 Pathophysiology 1311 Genotypic studies and gene expression arrays 1311 Immunophenotypic studies 1311 Clinical features 1312 Autoimmune phenomena 1312 Immunoglobulin deposition diseases 1312 Cryoglobulins 1313

Diagnosis and differential diagnosis 1313 Laboratory assessment 1313 Treatment 1313 Autoimmune phenomena 1314 Immunoglobulin deposition diseases 1314 Prognosis and long-term management 1314 Progression risk 1314 Overall survival 1315 Long-term management 1315 Plasmacytomas 1315 Introduction 1315 Diagnosis and investigation 1316 Treatment 1316 Solitary plasmacytoma of bone 1316 Solitary extramedullary plasmacytoma 1316 Prognosis 1316 Monoclonal immunoglobulin deposition disease 1317 Introduction 1317 Clinical features 1317 Diagnosis 1318 Management 1318 Key points 1321 References 1322

Plasma cell myeloma (PCM) is a plasma cell tumor with an incidence of 3-4 new cases per 100 000 population per annum, though this incidence demonstrates ethnic variation: the incidence is lowest in Chinese races at 1 per 100 000 and highest in European races at 4 per 100 000 and is commoner in Afro-American individuals with a male:female ratio of 3:2.1 It accounts for approximately 10 percent of hematologic malignancies and is characterized by a monoclonal proliferation of plasma cells which pro-

chain (paraprotein, M-protein, or M-component). The patient-specific paraprotein is present in the serum in all but 1-2 percent of patients who have nonsecretory disease. The median age at diagnosis is 65-70 years and there is a higher incidence in Afro-Caribbean ethnic groups.2