Dysproteinemia


Dysproteinemia

Description, Causes and Risk Factors:

An abnormality in plasma proteins (immunoglobulins).

Types may include: Anti-D immunoglobulin, chickenpox immunoglobulin, human normal immunoglobulin, immunoglobulin domains, immunoglobulin G subclass deficiency, measles immunoglobulin, monoclonal immunoglobulin, pertussis immunoglobulin, poliomyelitis immunoglobulin, rabies immunoglobulin, Rho(D) immunoglobulin, secretory immunoglobulin, secretory immunoglobulin A, selective immunoglobulin A deficiency, tetanus immunoglobulin, thyroid-stimulating immunoglobulins

Alternative Names May Be: Also called by the name familial idiopathic dysproteinemia, amyloid neuropathy.

Proteins are large molecules that consist of amino acids, sugars, and lipids. Albumin and globulin are two key components of protein. Albumin is made in the liver and constitutes approximately 60% of the total protein. The main function of albumin is to maintain osmotic pressure and to help transport certain blood constituents around the body via the bloodstream. Because albumin is made in the liver, it is one element that is used to monitor liver function.

Globulin is the basis for antibodies, glycoproteins (protein-carbohydrate compounds), lipoproteins (proteins involved in fat transport), and clotting factors. Globulins are divided into three main groups, the alpha-globulin, beta-globulin, and gamma-globulins. Alpha-globulins include enzymes produced by the lungs and liver, and haptoglobin, which binds hemoglobin together. The beta-globulins consist mostly of low-density lipoproteins (LDLs), substances involved in fat transport. All of the gamma-globulins are antibodies, proteins produced by the immune system in response to infection, during allergic reaction, and after organ transplants.

The relationship of albumin to globulin is determined by ratio (A/G), so when certain diseases cause the albumin levels to drop, the globulin level will be increased by the body in an effort to maintain a normal total protein level.

The body maintains a complex defense system with functional subunits. Immunoglobulins are one defensive force the body uses to protect against foreign invasions. Immunoglobulins constitute a part of body's serum proteins. Immunoglobulin carries antibodies which react with foreign bodies or antigens containing proteins.

Five distinct immunoglobulins are present in serum they may include, IgG, IgA, IgM, IgD, and IgE.

    IgG contains antiviral, antitoxic, and some antibacterial antibodies, it constitutes the major fraction of immunoglobulins.

  1. IgA carries a variety of antibodies and protects the mucous membranes surfaces as in the gastrointestinal and respiratory tracts, although it present in small amounts. IgA may be considered a front line in the body's defense against infectious material.

  2. IgM forms the natural antibodies such as the ABO blood group isoantibodies, rheumatoid factors, and antibodies of the gram-negative microorganism.

  3. IgD function is to signal the B cells to be activated. By being activated, they are ready to take part in the defense in the defense of the body in the immune system.

  4. IgE provide reaginic atopic, allergic, and anaphylactic antibodies.

All immunoglobulins are composed of four polypeptide chains liked by disulfide bonds. Two chains are heavy, the other two are light. The five types of heavy chains are gamma (?), alpha (?), mu (?), delta (?), and epsilon (-), from IgG, IgA, IgM, IgD, and IgE respectively. The two types of light chains are Kappa (K), and Lambda (3).

The five heavy chains and two light chains serve as a primary subunits for the body's immunoglobulin molecule.

Dysproteinemia is a heterogeneous disease characterized by abnormal, often excessive, synthesis of immunoglobulin (Ig) molecules or subunits. Dysproteinemia results from clonal proliferation of plasma cells or B lymphocytes. The abnormal circulating Ig molecules or subunits (most commonly free light chains) reach the glomerulus via the systemic circulation and are associated with the development of a variety of pathologic lesions within the kidney.

Free light chain molecules may pass through the glomerular basement membrane and form casts within distal tubular lumina (myeloma cast nephropathy) or form crystals within the cytoplasm of proximal tubules (light chain Fanconi syndrome). Alternatively, Ig molecules or subunits may form paraprotein tissue deposits and produce an array of pathologic lesions, most commonly amyloidosis and monoclonal Ig deposition disease.

The majority of cases are caused by plasma cell proliferations rather than B-cell lymphoproliferative disorders (LPDs), an alternative term for dysproteinemia is plasma cell dyscrasia (PCD).

Plasma cell dyscrasias are classified clinically, mainly on the basis of extrarenal parameters, including extent of plasmacytosis on bone marrow examination, the presence of an M spike on SPEP or UPEP, osteolytic lesions on skeletal survey, and hypercalcemia.

Symptoms:

Symptoms ofdysproteinemia typically start with smallfibre symptoms such as unpleasant sensory symptoms including pain; progressive sensory loss andmotor weakness occur later. Autonomic symptomsinclude nausea and vomiting, nocturnal diarrhea,postural lightheadedness and erectile dysfunction.Non-specific systemic features such as fatigue andweight loss are common.

Diagnosis:

dysproteinemia

A few clinical and pathologic points should be clarified before considering the individual disease entities.

First, the various forms of dysproteinemia-associated Renal disease may be the initial manifestation of a PCD or may occur in the setting of known MGUS (monoclonal gammopathies of undetermined significance) or MM (multiple myeloma).

Second, clinical features cannot distinguish among the various patterns of Renal disease associated with the dysproteinemias. Renal biopsy is necessary to establish the individual diagnoses and often provides important prognostic and therapeutic information.

Third, multiple patterns of dysproteinemia-associated Renal disease may coexist in the same biopsy sample. For instance, up to half of renal biopsies from patients with LCDD (light-chain deposition disease) exhibit coexistent findings of MCN (minimal-change nephropathy); in contrast, amyloidosis and MCN rarely coexist in the same biopsy sample. Importantly, the diagnosis of virtually all of the entities to be discussed is critically dependent on the inclusion of ? and ? in the standard panel of immunofluorescence stains. In some of the rarer entities, a more refined and precise diagnosis can be made with immunofluorescence staining for the subclasses of IgG. Collectively these stains may demonstrate light chain isotype restriction and ?-heavy chain subclass restriction, which strongly favors the presence of a monoclonal protein. That said, renal biopsy findings can not prove monoclonality, and additional techniques, including SPEP and UPEP (often with immunoelectrophoresis and immunofixation) are required.

All patients with suspected dysproteinemia should have serum and urine protein electrophoresis, immunoglobulins, immunofixation and light chains checked.

Other Tests:

Protein components tests measure the amounts and types of protein in the blood. Proteins are constituents of muscle, enzymes, hormones, transport proteins, hemoglobin, and other functional and structural elements of the body. Albumin and globulin make up most of the protein within the body and are measured in the total protein of the blood and other body fluids. Thus, the serum (blood) protein components test measures the total protein, as well as its albumin and globulin components in the blood.

Nerve conduction studies (NCS) typically show anaxonal length-dependent peripheral neuropathywhich is sensory more than motor. There is oftensuperimposed CTS (Carpal Tunnel Syndrome).Electromyography (EMG) maydemonstrate acute and chronic denervation withreinnervation. In the early stages of disease, NCSmay be normal but quantitative sensory testing maydemonstrate a small fibre neuropathy.

Treatment:

Dysproteinemia causes increased blood levels of abnormal proteins which interfere with the normal functioning of the blood platelets. Diseases like multiple myeloma (MM) and macroglobulinemia cause abnormal proteins to be released into the blood. The aim of treatment is to address the underlying cause and respective treatment.

Plasmapheresis, often in conjunction with other therapies, has been widely used to treat the dysproteinemias and their resulting clinical syndromes. Automated plasmapheresis, which separates plasma from the cellular blood elements by centrifugation, is used most commonly in the United States. Membrane separation and immunoadsorption techniques are more commonly used in Europe and Japan. In automated plasmapheresis, the plasma is removed from the patient's circulation and replaced with a protein-based fluid such as 5% human albumin solution or plasma protein fraction or with fresh frozen plasma. Membrane separation and immunoadsorption allow the offending proteins to be removed more selectively from the patient's plasma prior to the plasma being returned to the patient.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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