Paraproteinemia

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Dr mukhtar jama nour ,MBBSAmoud 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 .

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Three major disorders associated with paraproteinemia Monoclonal Gammopathy of

Undetermined Significance (MGUS) Multiple Myeloma Waldenströms Macroglobulinemia

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Waldenström's Macroglobulinemia(IgM paraprotein)

Lymphoplasmacytoid neoplasm

Tumor E ff ect s Paraprotein EffectsLymphadenopathySplenomegalyMarrow failure (esp anemia)

HyperviscosityNeuropathy

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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

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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.

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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

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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.

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fludarabine may be more effective in this disease. The monoclonal anti-CD20 antibody rituximab can also be effective. The median survival is 5 years .

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Stable Paraprotein at low concentration Not associated with marrow failure Not causing immunosuppression Not associated with bone lesions Marrow plasma cell numbers small

Monoclonal Gammopathy of UncertainSignificance (MGUS) (aka Benign Monoclonal Gammopathy)

Common in elderly (1-3% of population): approx 20% developmultiple myeloma by 15 years

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bone marrow failure pathological fractures hypercalcemia bone pain renal failure plasmacytoid lymphoma hyperviscosity

When to suspect a paraproteinemia

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If Patient is well No lymphadenopathy, splenomegaly Normal blood count Normal renal function Normal serum calcium Paraprotein is small

Make a provisional diagnosis of MGUS recheck the paraprotein concentration after a

few months

What to do when a paraprotein is discovered

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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

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THE END

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