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DR Medhat MRCP

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Paraproteinemia<br />

= monoclonal gammopathies<br />

= plasma cell dyscrasias<br />

- It`s the presence of excessive amounts of a single monoclonal gammaglobulin (in this<br />

case denominated "paraprotein") in the blood.<br />

- It is usually due to an underlying immunoproliferative disorder[i.e neoplasms of 1ry<br />

immune cells (B-cells,plasma cells,T-cells & NKC with excess production of Igs].<br />

• Types :<br />

- Paraproteinemias may be categorized according to the type of monoclonal protein<br />

found in blood:<br />

Light chains only (or Bence Jones protein). This may be associated with multiple<br />

myeloma or AL amyloidosis.<br />

Heavy chains only (also known as "heavy chain disease")<br />

Whole immunoglobulins. In this case, the paraprotein goes under the name of<br />

"M-protein" ("M" for monoclonal).<br />

- Most heavy chains & whole Ig remain in the blood vessels while light chains<br />

frequently escape & are excreted in urine (Bence Jones proteins)<br />

- Paraproteins may aggregate together in the blood to form macroglobulinemia which<br />

may lead to further complications e.g :<br />

Certain macroglobulins tend to precipitate within blood vessel with cold, a<br />

phenomenon known as cryoglobulinemia.<br />

Others may make blood too viscous to flow smoothly (usually with IgM<br />

macroglobulins), a phenomenon known as Waldenström macroglobulinemia.<br />

• Causes (differential diagnosis) of a serum paraprotein :<br />

Malignant :<br />

1. MM<br />

2. Lymphomas (usually B-cell NH- lymphoma with plasma cell component)<br />

3. Leukaemias : CLL , plasma cell leukemia.<br />

4. 1ry amyloidosis.<br />

Benign /stable :<br />

1. Monoclonal gammopathy of uncertain significance.<br />

2. Cold haemagglutinin disease.<br />

3. HIV .<br />

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