Plasma Cell Myeloma: Literature Review and Case Study

Abstract

1. Karen Ferreira, MS, MLS (ASCP)CM[⇑][1] 1. Department of Pathology, Hematology, Rhode Island Hospital, Providence, RI 1. Address for Correspondence: Karen Ferreira MS, MLS (ASCP)CM, Department of Pathology, Hematology, Rhode Island Hospital, 593 Eddy Street, Providence, RI 02903 401-444-4215, KAFerreira{at}Lifespan.org 1. Explain the pathophysiology of multiple myeloma. 2. Outline the different subsets of presentation. 3. Correlate the immunophenotype with the clinical and genetic aspects of myeloma. 4. List the results of the laboratory tests routinely evaluated in multiple myeloma. Introduction Plasma cell myeloma (multiple myeloma, myelomatosis, medullary plasmacytosis, Kahler's disease) is a B-cell neoplasm resulting from the expansion of a single clone of plasma cells (PC). The PCs synthesize a monoclonal immunoglobulin (paraprotein), a monoclonal immunoglobulin light chain (Bence-Jones protein) or both.1 The Bence-Jones proteins may lead to renal failure and the paraproteins can lead to peripheral blood (PB) hyperviscosity.2 Plasma cell myeloma (PCM) is clinically heterogeneous and may present with no symptoms or as an extremely aggressive disease.3 PCM is usually confined to the bone marrow (BM), with extramedullary involvement seen only in end stage cases.4,5 Epidemiology and Etiologies PCM constitutes 10-15% of all hematologic malignancies and 1% of all malignancies.6,7,8 PCM incidence increases with age with 40% of patients presenting under 60 years and only 2% occurring under the age of 40. The median age at diagnosis is 70. Incidence is greater in men than women (1.4:1), and occurs twice as frequently in African Americans than in Caucasians. The risk of PCM increases by 3.7 fold for individuals with a first degree relative with the disease.9,10 In 2007 approximately 20,000 new cases were diagnosed and 11,000 patients died from the disease.11,12 Clinical Features/Diagnostic Criteria The patients usually present with bone pain and pathologic fractures (due to lytic bone lesions). Anemia (due to bone marrow replacement of clonal PCs), recurrent infections (decreased normal immunoglobulin levels), and renal failure (Bence Jones deposition in kidneys) are common findings. Other clinical findings include hyperviscosity syndrome, hypercalcemia (increased osteoclastic activity), and epidural… ABBREVIATIONS: PCM – plasma cell myeloma, PC – plasma cell, PB – peripheral blood, BM – bone marrow, NCAM - neural cell adhesion mutation, VDC – cyclophosphamide, dexamethazone, bortezimib 1. Explain the pathophysiology of multiple myeloma. 2. Outline the different subsets of presentation. 3. Correlate the immunophenotype with the clinical and genetic aspects of myeloma. 4. List the results of the laboratory tests routinely evaluated in multiple myeloma. [1]: #corresp-1