Paraproteinemia

As paraproteinemia (Latin para - " beside", - emia - " in the blood " ) refers especially the frequent occurrence of a monoclonal immunoglobulin (so-called M- component) or monoclonal immunoglobulin light chains or the other proteins in the blood.

Subgroups

Among the sub-groups, each referring to a specific, is ascended in his concentration for different reasons over the physiological concentration range of protein include:

  • Multiple myeloma ( Syn: plasmacytoma )
  • IgG Deposition Disease
  • The osteoclastic Multiple myeloma ( POEMS )
  • The Heavy Chain Disease and
  • Monoclonal gammopathy of unknown significance, the ( MGUS ) and
  • Of Waldenstrom's macroglobulinemia.

These diseases are part of the group of non-Hodgkin's lymphomas.

Diagnostic criteria

From a Paraproteinemia one speaks, for example, if the proportion of plasma cells in the bone marrow cells exceeds 30%, or if it can be shown ( from existing plasma cells ) tumor in a tissue biopsy a plasmozytärer.

Another criterion is the M- gradient of the serum, which may indicate an immunoglobulin -induced paraproteinemia. This is the case when the concentration value of individual immunoglobulins exceeds certain values:

  • IgG at a concentration of> 35 g / l
  • In IgA concentration of> 20 g / l

In addition, a urine sample may shed light on a high protein content: If the concentration of one or more proteins in the urine to> 1 g/24h, one also starts from a paraproteinemia.

Diagnostic agents

For the determination of the above criteria, among others, protein electrophoresis, immunoelectrophoresis, immunofixation and quantitative Ig determination can be used ( both in serum and urine ). By measuring the serum free light chain in the proof of monoclonal immunoglobulin light chains is possible.

Signs of disease

Among the many symptoms of the disease include above all the hyperviscosity (hyper - " on ", viscous - " viscous " ) of the blood due to the increased number of proteins.

As a result of paraprotein can lead to amyloidosis, to neuropathies and coagulation disorders occur (due to the hyperviscosity ). Accompanying cold agglutinins may be detected. The lack of normal ( polyclonal ) immunoglobulins ( hypogammaglobulinemia ), increased susceptibility to infection exist.

Therapy

In the therapy of Paraproteinemias firstly the respective symptoms are treated.

To combat the cause of the addition, there are several different depending on the type of pathologically elevated protein treatment options. This includes the bisphosphonate treatment (with bone disease ), chemotherapy, immune modulation, stem cell transplantation, radiation therapy, and surgical procedures in which protein -forming tumors are surgically removed.

  • Hematology
  • Non -Hodgkin's lymphoma
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