Paraproteinemia

As paraproteinemia ( lat para -. "Next" , -ämie - "blood" ) is referred to the frequent occurrence of an in general or more specific proteins in the blood , which are therefore also referred to as Para proteins. Known forms of paraproteinemia are in particular monoclonal gammopathies , in which monoclonal immunoglobulins and / or free immunoglobulin light chains are present as paraprotein (also known as M component, M gradient or M protein due to the monoclonality). In some cases, the term paraproteinemia is also used synonymously for monoclonal gammopathy.

Associated clinical pictures

The clinical pictures to be classified as paraproteinemia, which each relate to a specific protein whose concentration has risen over the physiological concentration range for different reasons , include:

the Multiple Myeloma (obsolete: multiple myeloma )
the monoclonal gammopathy of unknown significance (MGUS)
the IgG Deposition Disease
the osteoclastic Multiple myeloma (POEMS)
the Heavy Chain Disease and
of Waldenstrom .

Some of these diseases belong to the group of non-Hodgkin lymphomas .

Diagnosis

There are no generally valid diagnostic criteria for paraproteinemia. In the case of monoclonal gammopathy, different limit values apply to define a certain clinical picture (such as multiple myeloma). To determine the paraprotein, protein electrophoresis , immunofixation electrophoresis and quantitative immunoglobulin determination (both in serum and urine) are used. The quantitative detection of monoclonal free immunoglobulin light chains is possible by measuring the free light chains in the serum.

Signs of illness

The various symptoms of the various paraproteinemias include, above all, the hyperviscosity (hyper - "over-" , viscous - "viscous" ) of the blood, which is caused by the increased number of proteins. As a result of paraproteinemia, amyloidosis , neuropathies and coagulation disorders (due to hyperviscosity) can occur. Cold agglutinins may also be detectable. The lack of normal (polyclonal) immunoglobulins ( hypogammaglobulinaemia ) can result in an increased susceptibility to infections. The various associated clinical pictures also show individually typical symptoms and characteristics that do not have to be found in every form of paraproteinemia.

therapy

In the therapy of paraproteinemia, on the one hand, the respective symptoms are treated. In order to further combat the cause, there are different therapy options depending on the type of pathologically elevated protein, which are also based on the malignancy of the disease. This includes bisphosphonate treatment (for bone disease), chemotherapy , immune modulation , stem cell transplantation , radiation therapy and surgical interventions in which protein-forming (solid) tumors may be surgically removed.

Individual evidence

^ ^A ^b SV Rajkumar et al .: International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma . In: Lancet Oncology . November 15, 2014, pp. E538 – e548. doi : 10.1038 / leu.2010.60 . PMID 20410922 .
↑ A Dispenzieri et al .: International Myeloma Working Group guidelines for serum-free light chain analysis in multiple myeloma and related disorders . In: Leukemia . 23, No. 2, November 20, 2008, pp. 215-224. doi : 10.1038 / leu.2008.307 . PMID 19020545 .
^ International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma
↑ SK Kumar et al. a .: Improved survival in multiple myeloma and the impact of novel therapies . In: Blood . No. 111 (5) , March 2008, pp. 2516-2520 , doi : 10.1182 / blood-2007-10-116129 .
↑ M. Gentile et al. a .: Emerging biological insights and novel treatment strategies in multiple myeloma . In: Expert Opinion on Emerging Drugs . No. 17 (3) , September 2012, p. 407-438 , doi : 10.1517 / 14728214.2012.713345 .
^ P. Moreau: The Future of Therapy for Relapsed / Refractory Multiple Myeloma: Emerging Agents and Novel Treatment Strategies . In: Seminars in Hematology . No. 49 (1) , July 2012, p. 33-46 , doi : 10.1053 / j.seminhematol.2012.05.004 .
↑ M. Dimopoulos et al .: Lenalidomide plus Dexamethasone for Relapsed or Refractory Multiple Myeloma . In: N Engl J Med . No. 357 , 2007, p. 2123-2132 ( abstract ).

[Rajkumar-1] A ^b SV Rajkumar et al .: International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma . In: Lancet Oncology . November 15, 2014, pp. E538 – e548. doi : 10.1038 / leu.2010.60 . PMID 20410922 .

[2] A Dispenzieri et al .: International Myeloma Working Group guidelines for serum-free light chain analysis in multiple myeloma and related disorders . In: Leukemia . 23, No. 2, November 20, 2008, pp. 215-224. doi : 10.1038 / leu.2008.307 . PMID 19020545 .

[3] International Myeloma Working Group (IMWG) Criteria for the Diagnosis of Multiple Myeloma

[Kumar_2008-4] SK Kumar et al. a .: Improved survival in multiple myeloma and the impact of novel therapies . In: Blood . No. 111 (5) , March 2008, pp. 2516-2520 , doi : 10.1182 / blood-2007-10-116129 .

[Gentile_2012-5] M. Gentile et al. a .: Emerging biological insights and novel treatment strategies in multiple myeloma . In: Expert Opinion on Emerging Drugs . No. 17 (3) , September 2012, p. 407-438 , doi : 10.1517 / 14728214.2012.713345 .

[Moreau_2012-6] P. Moreau: The Future of Therapy for Relapsed / Refractory Multiple Myeloma: Emerging Agents and Novel Treatment Strategies . In: Seminars in Hematology . No. 49 (1) , July 2012, p. 33-46 , doi : 10.1053 / j.seminhematol.2012.05.004 .

[7] M. Dimopoulos et al .: Lenalidomide plus Dexamethasone for Relapsed or Refractory Multiple Myeloma . In: N Engl J Med . No. 357 , 2007, p. 2123-2132 ( abstract ).