Multiple Myeloma (Part 1) - Update on Epidemiology, Diagnostic Criteria, Systemic Treatment and Prognosis

Guedes, Alex; Becker, Ricardo Gehrke; Teixeira, Luiz Eduardo Moreira

doi:10.1055/s-0043-1770149

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Update Article • Rev Bras Ortop 58 (3) • May-Jun 2023 • https://doi.org/10.1055/s-0043-1770149 copy

Multiple Myeloma (Part 1) - Update on Epidemiology, Diagnostic Criteria, Systemic Treatment and Prognosis

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Multiple myeloma (MM) is a hematological malignancy characterized by unregulated and clonal proliferation of plasma cells in the bone marrow; these cells produce and secrete an anomalous monoclonal immunoglobulin, or a fragment of this, called M protein. The clinical manifestations of MM result from the proliferation of these plasmocytes, the excessive production of monoclonal immunoglobulin and the suppression of normal humoral immunity, leading to hypercalcemia, bone destruction, renal failure, suppression of hematopoiesis and humoral immunity, increasing the risk for the development of infections. The increase in life expectancy of the world population led to a concomitant increase in the prevalence of MM, a pathology that usually affects the elderly population. The aim of this review is to update the reader on epidemiology, diagnostic criteria, differential diagnosis with other monoclonal gam-mopathies, systemic treatment and prognosis of MM.

epidemiology; monoclonal gammopathy of undetermined significance; multiple myeloma; smoldering multiple myeloma; plasmacytoma; prognosis

Disorder	Definition of the Disease
Monoclonal gammopathy of undetermined significance (Non-IgM MGUS)	All 3 criteria must be met: Serum monoclonal protein (Non-IgM) <3g/dl; Clonal plasma cells in the bone marrow <10%; ^a Absence of target organ damage, such as hypercalcemia, renal failure, anemia, or bone lesions (CRAB) that can be attributed to proliferative plasma cell disorder.
Latent Multiple Myeloma (SMM)	Both criteria must be met: Serum monoclonal protein (IgG or IgA) >3 g/dl, or monoclonal urinary protein >500mg in 24 hours and/or 10%-60% of clonal plasma cells in the bone marrow; Absence of events defining myeloma or amyloidosis.
Multiple Myeloma (MM)	Both criteria must be met: Clonal plasma cells in the bone marrow <10%orboneorextramedullaryplasmocytoma confirmed by biopsy; Any one or more of the events that define myeloma: Evidence of target organ damage that can be attributed to the underlying plasma cell proliferative disorder, specifically: Hypercalcemia: serum calcium >0.25 mmol/l (>1 mg/dl) higher than the upper limit of normal or >2.75 mmol/l (>11 mg/dl); Renal failure: creatinine clearance <40 ml per minute or serum creatinine > 177 mmol/l (>2 mg/dl); Anemia: hemoglobin value >2 g/dl below the lower limit of normal, ora hemoglobin value <10 g/dl; Bone lesions: one or more osteolytic lesions on skeletal radiographs, computed tomography (CT), or positron emission tomography-CT (PET-CT). Percentage of clonal plasma cells in the bone marrow >60%; Free serum light chain ratio (FLC) involved/uninvolved >100 (the level of free light chain involved should be >100mg/l); >1 focal lesion on magnetic resonance imaging (MRI) studies (at least 5 mm in size).
Monoclonal gammopathy of undetermined significance IgM (IgM MGUS)	All 3 criteria must be met: IgM monoclonal serum protein <3g/dl; Lymphoplasmocytic infiltration of the bone marrow <10% No evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly that can be attributed to the underlying lymphoproliferative disorder.
Light Chain MGUS	All criteria must be met: Abnormal FLC proportion (<0.26 or >1.65); Increased level of appropriate light chain involved (increased kappa FLC in patients with a ratio >1.65 and increased lambda FLC in patients with a ratio <0.26); There is no expression of heavy chain immunoglobulin in immunofixation; Absence of damage to target organs that can be attributed to the proliferative disorder of plasma cells; Clonal plasma cells in the bone marrow <10%; Monoclonal urinary protein <500 mg/24 hours.
Solitary Plasmocytoma	All 4 criteria must be met: Biopsy showing solitary lesion in bone or soft tissues, with evidence of clonal plasma cells; Normal bone marrow, no evidence of clonal plasma cells; Normal investigation of the skeleton, MRI (or CT) of the spine and pelvis (except for the primary solitary lesion); Absence of target organ damage such as hypercalcemia, renal failure, anemia, or bone lesions (CRAB) that can be attributed to lymphoplasmocyte proliferative disorder.
Solitary Plasmocytoma with Minimal Medullary Involvement ^b	All 4 criteria must be met: Biopsy showing solitary lesion in bone or soft tissues, with evidence of clonal plasma cells; Clonal plasma cells in the bone marrow <10%; Normal investigation of the skeleton, MRI (or CT) of the spine and pelvis (except for the primary solitary lesion); Absence of target organ damage such as hypercalcemia, renal failure, anemia, or bone lesions (CRAB) that can be attributed to lymphoplasmocyte proliferative disorder.

Determination of the patient's ISS stage
ISS Stage	Criteria
I	Serum ß2-microglobulin <3.5mg/l Serum albumin >3.5 g/dl
II	Not ISS I or ISS III Serum ß2-microglobulin >3.5mg/l, but <5.5mg/l or Serum albumin <3.5 g/dl
III	Serum ß2-microglobulin >5.5mg/l
Determination of risk according to chromosomal abnormalities detected by iFISH.
Risk	Criteria
Standard Risk	Chromosomal abnormalities without high risk
High Risk	Presence of del(17p), and/or t(4;14) translocation, and/or t(14,16) translocation
Determination of the risk according to the level of lactic dehydrogenase (LDH)
Risk	Criteria
Standard Risk	Normal serum LDH, < the upper limit of normal as defined by the laboratory
High Risk	High serum LDH, > the upper limit of normal as defined by the laboratory
Identification of the patient's R-ISS stage, according to the criteria determined above
R-ISS Stage	Criteria
I	ISS stage I and standard risk for chromosomal abnormalities by iFISH and normal DHL
II	Not R-ISS I or R-ISS III
III	ISS stage III and/or high risk for chromosomal abnormalities by iFISH or high DHL

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E-mail: rbo@sbot.org.br

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[1] Address for correspondence Alex Guedes, Rua Marechal Floriano 212/401, Canela, 40.110-010, Salvador, BA, Brazil (e-mail: alexguedes2003@yahoo.com.br).