Paraproteinemia is the presence of monoclonal gamma globulins in the blood, often caused by immunoproliferative disorders. Diagnosis is through serum protein electrophoresis and treatment involves managing the underlying cause.
Paraproteinemia is the presence of elevated levels of paraproteins or monoclonal gamma globulins in the blood, which is why it is also called monoclonal gammopathy. It usually occurs as part of immunoproliferative disorders such as leukemias, lymphomas, and plasma cell dyscrasias or cancers. This condition can also be a manifestation of a disease with no known cause, such as monoclonal gammopathy of undetermined significance. Diagnosis depends on detection of abnormal immune proteins in the blood, and treatment involves managing the underlying cause.
This condition occurs due to the presence of high amounts of a single monoclonal gamma globulin in the blood. A monoclonal gamma globulin, also called a paraprotein, comes from a single mother cell through multiple replicates; thus, paraproteins are essentially clones of each other. Monoclonal cells are characteristic of cancer cells. A type of B cell called a plasma cell produces gamma globulins, so an excess of monoclonal gamma globulins in the blood indicates an aberration in plasma cell replication. For this reason, leukemias and lymphomas, such as B-cell non-Hodgkin lymphomas, are expected to lead to paraproteinemia.
Gamma globulins have light chains and heavy chains, so paraproteinemias are classified into three types depending on which type of chain is predominant. Light chain paraproteinemia occurs in amyloid light chain (AL) amyloidosis. AL amyloidosis occurs in approximately 5-15% of patients with multiple myeloma. Heavy chain disease indicates immunoglobulin heavy chains. Gamma heavy chain disease or Franklin disease has fever, anemia, malaise, weakness, enlarged lymph nodes, and an enlarged liver and spleen.
The third type involves whole immunoglobulins, in which there is an abnormal ratio of light chains to heavy chains. In general, when monoclonal gammopathy involves heavy chains or whole immunoglobulins, the paraproteins are retained within the blood vessels. Sometimes, whole immunoglobulins clump together and form polymers, leading to a condition called macroglobulinemia. With macroglobulinemia, the blood becomes more viscous and tends to resist flowing, a condition that occurs in the disease called Waldenström’s macroglobulinemia or lymphoplasmacytic lymphoma.
When gammopathy involves light chains, paraproteins escape from blood vessels and are excreted by the kidneys. When a urine sample is examined under a microscope, it is seen as a Bence Jones protein. Bence Jones proteins can serve as a clue to the diagnosis of multiple myeloma or Waldenström’s macroglobulinemia.
Paraproteinemia is diagnosed through serum protein electrophoresis, which typically shows a peak or narrow band indicating overproduction of a certain protein. In electrophoresis, globulins are separated into alpha, beta and gamma bands. Paraproteins manifest as a peak in the gamma band. Treating this condition requires effective treatment of the underlying disorder, such as through chemotherapy or radiation therapy.
Protect your devices with Threat Protection by NordVPN
