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Disease definitions for the monoclonal gammopathies: MGUS and related disorders

Disease definitions for the monoclonal gammopathies: MGUS and related disorders
Type of monoclonal gammopathy Premalignancy with low risk of progression (1 to 2 percent per year) Premalignancy with high risk of progression (10 percent per year) Malignancy
IgG and IgA (non-IgM)*

Non-IgM MGUS

All criteria must be met:
  1. Serum monoclonal protein <3 g/dL
  2. Clonal BM plasma cells <10 percent
  3. Absence of end-organ damage such as CRAB that can be attributed to the PCPD  and absence of myeloma defining biomarkers

Smoldering MM

Both criteria must be met:
  1. Serum monoclonal protein (IgG or IgA) ≥3 g/dL and/or clonal BM plasma cells ≥10 percent
  2. Absence of end-organ damage such as CRAB that can be attributed to a PCPD and absence of myeloma defining biomarkers

MM

Clonal BM plasma cells ≥10 percent

Plus one of the following:
  1. Evidence of end-organ damage that can be attributed to the underlying PCPD, specifically one or more of the following:
    • Hypercalcemia: serum calcium ≥11.5 mg/dL
    • Renal insufficiency: serum creatinine >2 mg/dL or estimated creatinine clearance <40 mL/min
    • Anemia: normochromic, normocytic with hemoglobin >2 g/dL below lower limit of normal or hemoglobin <10 g/dL
    • Bone lesions: lytic lesions ≥5 mm attributed to a PCPD on skeletal radiography, MRI, CT, or PET/CT
  2. Presence of one of the following biomarkers:
    • ≥60 percent clonal plasma cells in bone marrow
    • Involved/uninvolved FLC ratio ≥100
    • MRI with >1 focal bone lesion
IgM

IgM MGUSΔ

All criteria must be met:
  1. Serum monoclonal protein <3 g/dL
  2. Clonal BM lymphoplasmacytic cells <10 percent
  3. Absence of end-organ damage such as anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly that can be attributed to the underlying lymphoproliferative disorder

Smoldering WM

Both criteria must be met:
  1. Serum IgM monoclonal protein ≥3 g/dL and/or BM lymphoplasmacytic infiltration ≥10 percent
  2. No evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly that can be attributed to the underlying lymphoproliferative disorder

WM

All criteria must be met:
  1. IgM monoclonal gammopathy (regardless of size of M protein)
  2. ≥10 percent BM lymphoplasmacytic infiltration (usual intertrabecular) by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation and a typical immunophenotype (eg, surface IgM+, CD5±, CD10, CD19+, CD20+, CD23) that satisfactorily excludes other lymphoproliferative disorders including chronic lymphocytic leukemia and mantle cell lymphoma
  3. Evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly that can be attributed to the underlying lymphoproliferative disorder

IgM myeloma

All criteria must be met:
  1. Symptomatic monoclonal PCPD characterized by a serum IgM monoclonal protein regardless of size
  2. Presence of ≥10 percent plasma cells on BM biopsy
  3. Presence of lytic bone lesions related to the underlying plasma cell disorder and/or translocation t(11:14) on FISH
Light chain

Light chain MGUS

All criteria must be met:
  1. Abnormal FLC ratio (<0.26 or >1.65)
  2. Increased level of the appropriate involved light chain (increased κ FLC in patients with ratio >1.65 and increased λ FLC in patients with ratio <0.26)
  3. No immunoglobulin heavy chain expression on immunofixation
  4. Clonal BM plasma cells <10 percent
  5. Absence of end-organ damage such as CRAB that can be attributed to the PCPD

Idiopathic Bence Jones proteinuria

All criteria must be met:
  1. Urinary monoclonal protein on urine protein electrophoresis ≥500 mg/24 h and/or clonal BM plasma cells ≥10 percent
  2. No immunoglobulin heavy chain expression on immunofixation
  3. Absence of end-organ damage such as CRAB that can be attributed to the PCPD

Light chain MMΔ

Same as MM except no evidence of immunoglobulin heavy chain expression on immunofixation
BM: bone marrow; CRAB: hypercalcemia, renal insufficiency, anemia, and bone lesions; FISH: fluorescent in situ hybridization; FLC: free light chain; MGUS: monoclonal gammopathy of undetermined significance; MM: multiple myeloma; PCPD: plasma cell proliferative disorder; WM: Waldenström macroglobulinemia.
* Occasionally, patients with IgD and IgE monoclonal gammopathies have been described and will be considered part of this category as well.
Δ Note that conventionally IgM MGUS is considered a subtype of MGUS, and similarly light chain MM is considered a subtype of MM. Unless specifically distinguished, when the terms MGUS and MM are used in general, they include IgM MGUS and light chain MM, respectively.
Modified with permission from: Rajkumar SV, Kyle RA, Buadi FK. Advances in the diagnosis, classification, risk stratification, and management of monoclonal gammopathy of undetermined significance: implications for recategorizing disease entities in the presence of evolving scientific evidence. Mayo Clin Proc 2010; 85:945. Copyright © 2010 Quadrant HealthCom, Inc.
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