Paraproteinemic hemoblastoses

Tumors, emerging from the cells, ensuring normal humoral immunity, combined into a group paraproteinemic haemoblast. As it is known, antibody synthesis (immunoglobulins) normally carried System polyclonal plasma cells and lymphocytes, which can react with any antigen. Each cell clone capable of synthesizing only one species of antibody, t. it is. odnorodnyi immunoglobulin. The immunological response to antigenic stimuli multiple cells are involved many clones.

By definition, the WHO expert committee, to immunoglobulins are proteins of animal origin, which may have specific activity of antibody, as well as proteins, similar to them in chemical structure and. Consequently, by antigennoj specifics.

There are five classes of immunoglobulins:

• IgG immunoglobulin;
• Immunoglobulin IgA;
• Immunoglobulin IgM;
• Immunoglobulin IgD;
• Immunoglobulin IgE.

They differ in the physiological, chemical and biological properties. Immunoglobulins are a part of the whey proteins; 90-95 % blocked consists of proteins with a molecular weight 160000-200000, 5-10 % macroglobulins up with a molecular weight of 900000-1000000.

Each immunoglobulin molecule consists of four polypeptide chains, two of which have a molecular weight of 50000-70000 and are called heavy (H – heavy), two - with a molecular mass of 20000-25000 and called light (L – light) chains.

On antigenic properties are five types of heavy chains: c, a, m, и d e, and two types of light: и x L.

When paraproteinemic hemoblastoses tumor cells genetically uniform, because, according to the theory of clonal, they are the descendants of one, somatic cell changes (plasma or lymphoid). Production of these cells is also uniform and is monoklonovy paraimmunoglobulin. By analogy with normal immunoglobulins paraimmunoglobuliny (PIg) also divided into five classes: QUILL, Hitting, Pigm, PIgD, Pigm.

Each of the classes are divided into subclasses. Incomplete synthesis of heavy chain paraproteins appears in the urine of patients with light chain product - mikromolekuljarnыj ʙelok-Benz Dƶonsa, which is easy to pass through the renal filter. Recent research proved the structural identity of normal immunoglobulins and paraproteins, also established, that some proteins are active couple antibodies to natural human antigens, animal and synthetic substances - haptens. But accumulation PIg in the body - a pathological phenomenon, because it leads to severe disorders of the various organ systems.

Currently, the group paraproteinemic Leukemia include three major diseases:

• myeloma (plasmacytoma);
• Waldenstrom's disease (makroglobulinemichesky lymphocytosis);
• heavy chain disease.