(Dysproteinaemias; monoclonal gammopathies; Paraproteinemias; plasma cell dyscrasias)

Plasma cell diseases are a heterogeneous group of diseases of unknown etiology which is characterized by disproportionate proliferation of a B cell clone presence of a structurally and electrophoretically homogeneous (monoclonal) immunoglobulins or polypeptide subunit in serum and / or urine pathophysiology (Structural features and classification of the immunoglobulins, components of the immune system: antibody). After their development in the bone marrow undifferentiated B cells pass over (z. B. Peyer’s patches) in peripheral lymphoid tissue such as lymph nodes, spleen and intestines. Here they begin to differentiate into cells that can respond to a limited number of antigens. After contact with the corresponding antigen, some B-cells undergo clonal proliferation into plasma cells. Each clonal plasma cell line is intended to synthesize a specific immunoglobulin antibody of 2 identical heavy chains (gamma [?], my [?], alpha [?] delta [?] or epsilon [?]) and 2 identical light chains is (kappa [?] or lambda [?]). Normally, there is a slight excess of light chains, and the urinary excretion of small amounts of free light chain polyclonal (? 40 mg / 24 h) is normal. Plasma cell diseases have an unclear etiology and are characterized by the disproportionate proliferation of one clone. The consequence is a corresponding increase in the serum levels of their product, the monoclonal immunoglobulin (paraprotein). Paraproteins may consist of heavy and light chains or only of a chain type. The complications of plasma cell proliferation and M-protein production include the following: autoimmune damage of organs (especially the kidneys): some M proteins show antibody activity Impaired immunity: Decreased production of other immunoglobulins bleeding tendency: M proteins can coat platelets, inactivate clotting factors increase blood viscosity, and by other mechanisms cause bleeding Secondary amyloidosis: M-protein can be deposited within organs osteoporosis, hypercalcemia, anemia or pancytopenia: Cloned cells, the bone matrix and / or the bone marrow infiltrate plasma cell diseases ranging from asymptomatic, stable states (in which only the detectable paraprotein) to progressive malignant tumors (such as multiple myeloma, for the classification see Table:. classification of Plasmazellkrankh nits). Rarely, transient plasma cell disease found in patients with drug hypersensitivity (sulfonamides, phenytoin, and penicillin), with suspected viral infections and after heart operations or transplants. Classification of plasma cell disease symptoms Description Examples Monoclonal gammopathy of undetermined significance * Asymptomatic, usually not progressive occurrence in apparently healthy persons or in those with other conditions in conjunction with non-lymphoreticular tumors breast, bile duct, Gastrointestinaltrakt-, kidney and prostate cancers in conjunction with chronic inflammatory and infectious conditions Chronic cholecystitis, osteomyelitis, pyelonephritis, rheumatoid arthritis, tuberculosis In combination with various other diseases Familial hypercholesterolemia, Gaucher’s disease, thyrotoxicosis, Kaposi’s sarcoma, lichen myxoedematosus, liver disease, myasthenia gravis, pernicious anemia malignant plasma cell disease Asymptomatic, progressive Usually, only light chains (Bence Jones ) but occasionally full immunoglobulin molecules (IgG, IgA, IgM, IgD) Early multiple myeloma Symptomatic, progressive Excessive production of IgM macroglobulinemia most frequently IgG, IgA, or only light chains (Bence Jones) Multiple myeloma usually only light chains (Bence Jones), but occasionally full immunoglobulin molecules (IgG, IgA, IgM, IgD) Nichtheredit√§re primary systemic amyloidosis heavy chain diseases IgG heavy chain (?-chain) disease (occasionally benign) IgA heavy chain disease IgM heavy chain disease IgD-heavy chain disease Vorpbergehende plasma cell disorders Not necessarily symptomatic in connection with a drug hypersensitivity, viral infections and heart operations or transplants hypersensitivity to sulfonamide, phenytoin or penicillin * Age-related incidences. If diagnosis based on clinical manifestations, as part of the investigation of anemia or because of incidental findings (elevated serum proteins or proteinuria) of suspected plasma cell disease arises, further investigations are initiated by serum or Urineiwei√üelektrophorese. The detected by electrophoresis paraprotein is further examined by immunofixation for identifying heavy or light chain classes.

Comments

Leave a Reply

Sign In

Register

Reset Password

Please enter your username or email address, you will receive a link to create a new password via email.