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    Monoclonal Gammopathy of Unknown Significance (MGUS) : George Herman Hariman

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    Abbi Yanto Art
    This document summarizes updated definitions and classifications for monoclonal gammopathy of unknown significance (MGUS), smoldering multiple myeloma (SMM), multiple myeloma (MM), and solitary plasmacytoma by the International Myeloma Working Group. It defines the key criteria for each, including serum and urine monoclonal protein levels, bone marrow plasma cell percentage, and presence of end organ damage. Diagnostic guidelines around tests like serum free light chain ratios, bone marrow biopsy vs aspiration, and imaging are also provided.

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    Monoclonal Gammopathy of Unknown Significance (MGUS) : George Herman Hariman

    Uploaded by

    Abbi Yanto Art
    52 views30 pages
    This document summarizes updated definitions and classifications for monoclonal gammopathy of unknown significance (MGUS), smoldering multiple myeloma (SMM), multiple myeloma (MM), and solitary plasmacytoma by the International Myeloma Working Group. It defines the key criteria for each, including serum and urine monoclonal protein levels, bone marrow plasma cell percentage, and presence of end organ damage. Diagnostic guidelines around tests like serum free light chain ratios, bone marrow biopsy vs aspiration, and imaging are also provided.

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    MGUS

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    MGUS

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    This document summarizes updated definitions and classifications for monoclonal gammopathy of unknown significance (MGUS), smoldering multiple myeloma (SMM), multiple myeloma (MM), and solitary plasmacytoma by the International Myeloma Working Group. It defines the key criteria for each, including serum and urine monoclonal protein levels, bone marrow plasma cell percentage, and presence of end organ damage. Diagnostic guidelines around tests like serum free light chain ratios, bone marrow biopsy vs aspiration, and imaging are also provided.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    52 views30 pages

    Monoclonal Gammopathy of Unknown Significance (MGUS) : George Herman Hariman

    Uploaded by

    Abbi Yanto Art
    This document summarizes updated definitions and classifications for monoclonal gammopathy of unknown significance (MGUS), smoldering multiple myeloma (SMM), multiple myeloma (MM), and solitary plasmacytoma by the International Myeloma Working Group. It defines the key criteria for each, including serum and urine monoclonal protein levels, bone marrow plasma cell percentage, and presence of end organ damage. Diagnostic guidelines around tests like serum free light chain ratios, bone marrow biopsy vs aspiration, and imaging are also provided.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 30

    Monoclonal Gammopathy of

    Unknown Significance
    (MGUS) George Herman
    Hariman
    Div of Haematology
    Dept of Clinical Pathology
    School of Medicine
    University of North Sumatera
    It is important to define MGUS

     As it does not need treatment


     It is not cancer
     But it may have a risk to get blood disease (?)
    and infection
     Only follow up is needed
    International Myeloma Working
    Group

     MM (Multiple Myeloma) Updated definition


     SMM (Smouldering Multiple Myeloma)
    Updated defintion
     MGUS (Monoclonal Gammopathy of
    Undetermined Significance) Updated
    classification
     Solitary Plasmacytoma Updated definition and
    classification
    Important notes

     MGUS diagnosis: BMB (biopsy) can be deferred if


    patients appear to be at low risk of MGUS
     SMM diagnosis follow up recommendation MRI
    (whole body or spine), whole body-CT or PET-CT are
    needed at baseline in patients considered to have
    SMM
     BMPC (bone marrow plasma cell) estimation
    – either aspirate or biopsy can be used to estimate BMPC
    – If values are discrepant (aspirate or biopsy) use the highest
    – Estimation of BMPC by flowcytometry is not recommended
    Updated defintion of MM-1

     In addition to CRAB the following MDEsare


    considered to MM
    – CRAB: Calcium (elevated), Renal failure, Anaemia,
    Bone Lesion.
    – MDEs (Myelome Defining Events): CRAB + one of 3
    features
     BMPC ≥ 60%
     Srum FLC (free light chain) ratio ≥ 100 provided involved
    FLC ≥ 100 mg/dl
     > 1 focal lesion on MRI
    CRAB

     Hypercalcemia: serum Ca++ > 11 mg/dl


    (>2.75 mmol/L)
     Renal failure: Creatinin clearance < 40 mg/min
    or serum creatinin > 2mg/dl (177 umol/L)
     Anaemia: Hb < 10 g/dl or difference of > 2 g/dl
    from the normal local lower limit Hb value.
     Bone Lesion ≥1 osteolytic lesion on skeletal
    radiography, CT or PET-CT
    Updated defintion of MM-2
     CT and PET-CT can be used for detecting osteolytic bone lesions
     Creatinin clearance of < 40 ml/min can be used as cut-off point of
    renal failure in addition to serum creatinin (> 2mg/dl)
     Only suspected or biopsy proven light chain cast nephropathy
    allowed as renal MDE
     Presence of M-protein is not requiered for diangsosis of MM and
    only to classify MM into secretory and non-secretory type
     Osteoporosis, compression fracture, infection, hyperviscosity
    syndrome and peripheral neuropathy alone are not considered as
    MDEs
    Updated definition of SMM

     ≥ 3 g/dl serum M-protein or ≥ 200 mg/24 hr


    urine M protein and/or ≥ 10% BMPC plus no
    MDEs or AL amyloidosis (Immunolightchain
    Amyloidosis)
    Updated classification of MGUS

     Non-IgM MGUS
     IgM MGUS
     Light Chain MGUS
    Non-IgM MGUS (all must be
    present)

     Serum monoclonal protein (Non-IgM type) <


    3g/dl
     Clonal BMPC < 10%
     No end-organ damage such as CRAB that can
    be attributed to the plasma cell proliferative
    disorder
    IgM MGUS (all must be present)

     Serum monoclonal protein (IgM type) > 3g/dl


     Clonal BMPC < 10%
     No end-organ damage such as CRAB that can
    be attributed to the plasma cell proliferative
    disorder
    Light chain MGUS (all criteria must
    be met)

     Abnormal FLC (<0.26 or >1.65)


     BMPC < 10%
     No end-organ damage such as CRAB that can
    be attributed to the plasma cell proliferative
    disorder
    Immunoglobulin molecule
    Antigen
    binding site Disulfide
    bond

    Light chain
    Plasma
    cell
    B-Cell Hinge region

    Heavy chain

     FLC dimers
    
    FLC monomers
    FLC monomers
    Free Light Chain Immunoassay
     / > 1.65 involved FLC is Kappa () and
    the uninvolved is Lambda

     / < 0.26 involved FLC is Lambda () and


    the univolved is Kappa

     Serum involved:univolved FLC Ratio


    ≥100% (and the involved FLC must be
    measurable ≥ 100 mg/L
    Updated classification and
    defintion of Solitary Plasmacytoma

     Type-1: No clonal BMPC


     Type-2: with minimal marrow involvement
    (clonal BMPC present byt < 10%)

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