Plasma cell diseases: causes, symptoms, diagnosis, treatment

Plasma cell diseases (dysproteinemia, monoclonal gammopathies, paraproteinemia, plasma cell discourses) are a group of diseases of unknown etiology characterized by a disproportional proliferation of one B cell clone, the presence of structurally and electrophoretically homogeneous (monoclinic) immunoglobulins or polypeptides in blood serum or urine.

[1], [2]

Causes of the plasma cell diseases

The etiology of plasma cell diseases is unknown, they are characterized by a disproportionate proliferation of one clone. The result is a corresponding increase in the serum level of their product - monoclonal immunoglobulin (M-protein).

M-protein can have in its composition, both heavy and light chains, or only one type of chain. Antibodies show a certain activity, which can lead to autoimmune damage to organs, especially the kidneys. In the production of M-protein, the production of other immunoglobulins usually decreases and immunity is thus impaired. M-protein is able to coat platelets, inactivate clotting factors, increase blood viscosity and, in addition, provoke bleeding by other mechanisms. M-protein can cause secondary amyloidosis. Clonal cells often infiltrate the matrix of bones and bone marrow, leading to osteoporosis, hypercalcemia, anemia and pancytopenia.

[3], [4]

Pathogenesis

After appearance in the bone marrow, undifferentiated B-cells migrate to the peripheral lymphoid tissues: lymph nodes, spleen, intestine and Peyer's plaques. Here they begin to differentiate into cells, each of which is capable of responding to a limited number of antigens. After meeting with the corresponding antigen, some B cells undergo clonal proliferation into plasma cells. Each clonal line of plasma cells is capable of synthesizing one specific antibody - an immunoglobulin consisting of one heavy chain (gamma, mu, alpha, epsilon or delta) and one light chain (kappa or lambda). Normally, slightly more light chains are produced, and urinary excretion of a small number of free polyclonal light chains (<40 mg / 24 h) is normal.

[5], [6], [7]

Symptoms of the plasma cell diseases

Plasma cell diseases range from asymptomatic, stable conditions (in which only a protein is recorded) to progressive neoplasia (eg, multiple myeloma). Occasionally, transient plasma cell diseases are associated with drug hypersensitivity (sulfonamides, phenytoin, penicillin), viral infections and surgical interventions on the heart.

Forms

Category	Symptoms	Disease	Comments and examples
Monoclonal gammopathy of undetermined character	Bessim-ptomnaya Usually not progressing	Associated with non-lymphoreticular tumors Associated with chronic inflammatory and infectious conditions Associated with various other diseases	Mainly carcinomas of the prostate, kidney, GIT, mammary gland and bile ducts Chronic cholecystitis, osteomyelitis, tuberculosis, pyelonephritis, RA Myxedema lehen, liver disease, thyrotoxicosis, pernicious anemia, myasthenia gravis, Gaucher's disease, familial hypercholesterolemia, Kaposi's sarcoma Can occur in relatively healthy people; with age more often
Malignant plaque diseases	There are symptoms of the disease, a progressive course	Macroglue-bouillon Multiple myeloma Non-hereditary primary systemic amyloidosis Disease of heavy chains	IgM Most often IgG, IgA or only light chains (Bence-Jones) Normally, only light chains (Bence-Jones), but sometimes intact molecules of immunoglobulins (IgG, IgA, IgM, IgD) IgG disease of heavy chains (sometimes benign). IgA heavy chain disease. IgM disease of heavy chains. IgD heavy chain disease
Transitory plasma cell diseases			Associated with drug hypersensitivity, viral infections and surgical interventions on the heart

[8], [9], [10]

Diagnostics of the plasma cell diseases

Suspicion of the presence of plasma-cell disease occurs when there is a clinical manifestation (often anemia), an increased level of whey protein or proteinuria, which gives grounds for further examination with the electrophoresis of blood serum or urine proteins where M protein is found. M-protein is subjected to further analysis with the implementation of immunofixing electrophoresis to identify classes of heavy and light chains.

[11], [12], [13], [14], [15], [16]