Ebook Handbook of hematologic malignancies: Part 2
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Ebook Handbook of hematologic malignancies: Part 2
https://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath Sundara Ebook Handbook of hematologic malignancies: Part 2arajan, Abhijeet Kumar,Amit Agarwal, and Neha KordeINTRODUCTIONPlasma cell (PC) dyscrasias are disorders characterized by clonal proliferation of PCs with or without elevated levels of monoclonal protein (M protein) and immunoglobulin fragments. It encompasses a number of disorders including monoclo Ebook Handbook of hematologic malignancies: Part 2nal gammopathy of unknown significance (MGUS), smoldering myeloma (SMM), solitary plasmacytoma (SP), multiple myeloma (MM), PC leukemia, Waldenstrom'sEbook Handbook of hematologic malignancies: Part 2
macroglobulinemia (WM), and amyloidosis (AL). In this chapter, we focus on MGUS, SMM, and plasmacytoma (see Chapters 26-28).MONOCLONAL GAMMOPATHY OF https://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath Sundara Ebook Handbook of hematologic malignancies: Part 2h a prevalence in the United States estimated to be slightly greater than 3% in Whites who are 50 or older. In a population-based study from Olmstead County, Minnesota, the annual incidence of MGUS was noted to be 120 per 100,000 and 60 per 100,000 in men and women greater than 50 years, respectivel Ebook Handbook of hematologic malignancies: Part 2y (Mayo Clin Proc. 2010;85(10):933-942). In men and women greater than 90 years, the incidence of MGUS increased to 530 per 100,000 and 370 per 100,00Ebook Handbook of hematologic malignancies: Part 2
0, respectively.DIAGNOSISThe 2014 International Myeloma Working Group (IMWG) definition for MGUS is summarized in Table 25.1.1 By definition, patientshttps://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath Sundara Ebook Handbook of hematologic malignancies: Part 2enefit, health care burden, and psychological implications, universal screening for MGUS is not advocated. The workup for MGUS needs to incorporate the tests to rule out more serious conditions, such as SMM or MM (Figure 25.1). Bone mar-rn\A/ ÍRI\/n hinncv ran hr rnneidprod in natinntc \A/hn ana Inc Ebook Handbook of hematologic malignancies: Part 2e than148 PLASMA CELL DISORDERSTable 25.1 IMWG DefinitionsMGUS (non-IgM)•Serum non-IgM M protein <3 g/dL•Clonal BM PCs <10%•*Absence of end organ damaEbook Handbook of hematologic malignancies: Part 2
ge, such as hypercalcemia, renal insufficiency, anemia, and bone lesions (CRAB features) or AL that can be attributed to a plasma cell disorderMGUS(lghttps://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath Sundara Ebook Handbook of hematologic malignancies: Part 2enopathy, hepatosplenomegaly, or other end organ damage that can be attributed to the underlying lymphoproliferative disorderSmoldering myeloma•Serum M protein (IgG or IgA) >3 g/dL or urinary M protein >500 mg per 24 hr and/or clonal BM plasma cells 10%-60%•*Absence of end organ damage (CRAB), myelo Ebook Handbook of hematologic malignancies: Part 2ma-defining events** or ALSolitary plasmacytoma•Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells•Clonal boneEbook Handbook of hematologic malignancies: Part 2
marrow plasma cells <10%•Normal skeletal survey and MRI (or CT) of spine and pelvis (except for the primary solitary lesion)•’ Absence of end-organ dahttps://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath Sundara Ebook Handbook of hematologic malignancies: Part 2 bone lesions; IgA, immunoglobulin A; IgG, immunoglobulin G; IgM, immunoglobulin M; IMWG, International Myeloma Working Group; MGUS, monoclonal gammopathy of unknown significance; M protein, monoclonal protein; PC, plasma cell.*Evidence of end organ damage that can be attributed to the underlying PC Ebook Handbook of hematologic malignancies: Part 2 proliferative disorder (hypercalcemia: serum calcium >1 mg/dL higher than the upper limit of normal or >11 mg/dL; renal insufficiency: creatinine cleEbook Handbook of hematologic malignancies: Part 2
arance <40 mưmin or serum creatinine >2 mg/dL; anemia: hemoglobin value of 2 g/dL below the lower limit of normal or <10g/dL; one or more osteolytic lhttps://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath Sundara Ebook Handbook of hematologic malignancies: Part 2ee light chain ratio >100; (c) >1 focal lesions on MRI studies.Source: Rajkumar et al.1Based on the immunoglobulin heavy chain and light chain involved, MGUS can be subclassified into three different types.1Non-IgM MGUS—Immunoglobulin G (IgG), immunoglobulin25. MGUS, SMM, AND PLASMACYTOMAS 149 Ebook Handbook of hematologic malignancies: Part 2https://khothuvien .comPLASMA CELL DISORDERSMonoclonal Gammopathy of Unknown o IT Significance, Smoldering Myeloma,53 and PlasmacytomasSrinath SundaraGọi ngay
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