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    Mohamoud Mohamed
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    This document discusses paraproteinemia, which is the presence of a monoclonal immunoglobulin or immunoglobulin light chain in the blood or urine resulting from plasma cell or B-lymphocyte clonal proliferation. It specifically focuses on Waldenstrom's macroglobulinemia, which is a rare low-grade lymphoma associated with an IgM paraprotein that causes hyperviscosity syndrome. The key symptoms are features of hyperviscosity like nosebleeds and bruising. Diagnosis involves finding an IgM paraprotein and bone marrow infiltration by lymphoid cells. Treatment may require plasmapheresis and chemotherapy or rituximab can be effective, with median survival being 5 years.

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    This document discusses paraproteinemia, which is the presence of a monoclonal immunoglobulin or immunoglobulin light chain in the blood or urine resulting from plasma cell or B-lymphocyte clonal proliferation. It specifically focuses on Waldenstrom's macroglobulinemia, which is a rare low-grade lymphoma associated with an IgM paraprotein that causes hyperviscosity syndrome. The key symptoms are features of hyperviscosity like nosebleeds and bruising. Diagnosis involves finding an IgM paraprotein and bone marrow infiltration by lymphoid cells. Treatment may require plasmapheresis and chemotherapy or rituximab can be effective, with median survival being 5 years.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
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    48 views14 pages

    Para Protein Emi A

    Uploaded by

    Mohamoud Mohamed
    This document discusses paraproteinemia, which is the presence of a monoclonal immunoglobulin or immunoglobulin light chain in the blood or urine resulting from plasma cell or B-lymphocyte clonal proliferation. It specifically focuses on Waldenstrom's macroglobulinemia, which is a rare low-grade lymphoma associated with an IgM paraprotein that causes hyperviscosity syndrome. The key symptoms are features of hyperviscosity like nosebleeds and bruising. Diagnosis involves finding an IgM paraprotein and bone marrow infiltration by lymphoid cells. Treatment may require plasmapheresis and chemotherapy or rituximab can be effective, with median survival being 5 years.

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
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    of 14

    Paraproteinemia

    6/30/2014 1
    Dr mukhtar jama nour ,MBBS
    Amoud university for health and science
    institute
    Definition of a paraprotein
    A monoclonal immunoglobulin or
    immunoglobulin light chain in the blood or
    urine resulting from a clonal proliferation of
    plasma cells or B-lymphocytes .
    6/30/2014 2
    Three major disorders
    associated with paraproteinemia
    Monoclonal Gammopathy of Undetermined
    Significance (MGUS)
    Multiple Myeloma
    Waldenstrms Macroglobulinemia
    6/30/2014 3
    Waldenst rm's Macroglobulinemia
    (IgM paraprot ein)
    Lymphoplasmacytoid neoplasm
    Tumor Effect s Paraprot ein Effects
    Lymphadenopat hy
    Splenomegaly
    Marrow failure (esp anemia)
    Hyperviscosit y
    Neuropat hy
    6/30/2014 4
    Waldenstroms macroglobulinemia
    This is a low-grade lymphoplasmacytoid
    lymphoma associated with an IgM
    paraprotein, causing clinical features of
    hyperviscosity syndrome. It is a rare tumour
    occurring in the elderly and affects a slight
    excess of males
    6/30/2014 5
    Clinical presentations
    Patients classically present with features of
    hyperviscosity, such as nosebleeds, bruising,
    confusion and visual disturbance. However,
    presentation may be with anaemia, systemic
    symptoms, splenomegaly or
    lymphadenopathy.
    6/30/2014 6
    Patients are found on investigation to have
    an IgM paraprotein associated with a raised
    plasma viscosity. The bone marrow has a
    characteristic appearance, with infiltration of
    lymphoid cells and prominent mast cells

    6/30/2014 7
    Management

    Severe hyperviscosity and anaemia may
    necessitate plasmapheresis to remove IgM
    and make blood transfusion possible.
    Treatment with alkylating agents, such as
    chlorambucil, is effective but rather slow.
    6/30/2014 8
    fludarabine may be more effective in this
    disease. The monoclonal anti-CD20 antibody
    rituximab can also be effective. The median
    survival is 5 years .
    6/30/2014 9
    St able Paraprot ein at low concent rat ion
    Not associat ed wit h marrow failure
    Not causing immunosuppression
    Not associat ed wit h bone lesions
    Marrow plasma cell numbers small
    Monoclonal Gammopat hy of Uncert ain
    Significance (MGUS)
    (aka Benign Monoclonal Gammopat hy)
    Common in elderly (1-3%of populat ion): approx 20%develop
    mult iple myeloma by 15 years
    6/30/2014 10
    bone marrow failure
    pat hological fract ures
    hypercalcemia
    bone pain
    renal failure
    plasmacyt oid lymphoma
    hyperviscosit y
    When t o suspect a paraprot einemia
    6/30/2014 11
    If
    Pat ient is well
    No lymphadenopat hy, splenomegaly
    Normal blood count
    Normal renal funct ion
    Normal serum calcium
    Paraprot ein is small
    Make a provisional diagnosis of MGUS
    recheck t he paraprot ein concent rat ion aft er a
    few mont hs
    What t o do when a paraprot ein is discovered
    6/30/2014 12
    After follow-up of 20 years, only one-quarter
    will progress to myeloma or a related
    disorder. There is no way of predicting
    progression in an individual patient, and if
    investigations remain stable, annual
    monitoring is all that is required
    6/30/2014 13
    THE END
    6/30/2014 14

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