System mastocytosis
Contents:
Description:
The system mastocytosis - a rare disease, is observed preferential at adults. The skin mastocytosis, or a pigmental small tortoiseshell, meets considerably more often, is preferential at children.
Symptoms of the System mastocytosis:
The system mastocytosis is characterized by abnormal infiltration by mast cells not only skin, but also marrow, a liver, a spleen, digestive tract and other internals. In rare instances the disease proceeds without damage of skin. At a part of patients the system mastocytosis is associated with development of a hematologic picture of other hemoblastosis, is more often than a chronic myeloproliferative disease (a subleukemic myelosis, a chronic miyelomonotsitarny leukosis), is more rare – a zrelokletochny limfoproliferation or a miyelodisplastichesky syndrome.
The clinical option of a system mastocytosis proceeding with massive damage of marrow (20% of mastocytes) and emergence of abnormal mast cells in blood (in typical cases of 10%), is designated as a tuchnokletochny leukosis. This option differs in the lack of skin defeats and an adverse current which is quickly leading to development of multiorgan insufficiency and the death of patients.
In a clinical picture of a system mastocytosis it is necessary to allocate 2 groups of symptoms. The first are caused by infiltration by mast cells of bodies and fabrics and, respectively, are expressed in increase in data of bodies and/or disturbance of their function. The second group of symptoms is connected with functional activity of mastocytes, namely synthesis and release of biologically active products among which heparin and a histamine have the most important physiological value. Treat the second group of symptoms: 1) ntoksikation (fever, profuse sweats, loss of weight); 2) the attacks like inflows arising spontaneously after insolation or as a result of provocative medicamentous influences (for example, an anesthesia); 3) skin itch; 4) osteoporosis or osteofibrosis; 5) diarrhea and cankers of digestive tract; 6) the hemorrhagic syndrome which is the most frequent deadly complication.
Clinical current of a mastocytosis, as a rule, long, slowly progressing. Patients for many years are left in the compensated state without treatment. A pigmental small tortoiseshell can progress in a system mastocytosis. The nature of manifestations and a current of a system mastocytosis varies from high-quality slow to the aggressive forms which are quickly leading to fatal dysfunction of internals or a heavy hemorrhagic syndrome. Transformation in a tuchnokletochny leukosis is occasionally observed.
Reasons of the System mastocytosis:
Mastocytosis – a disease of the tumoral nature. The etiology and a pathogeny are not established.
Treatment of the System mastocytosis:
As symptomatic therapy inhibitors of products and antagonists of the mediators which are released from mast cells are used namely: H1 and H2-antihistaminic drugs, stabilizers of membranes of mast cells. Use of these drugs is effective only at patients with a pigmental small tortoiseshell and a high-quality current of a system mastocytosis. At patients with an aggressive current of a mastocytosis effective methods of therapy are not developed. There are separate messages on positive takes of use of drugs of alpha interferon (3 mln units x 3 times a week subcutaneously) and cyclosporine A (300 mg/day) in combination with small doses of Methylprednisolonum (5-10 mg/day).
Efficiency of treatment is determined by a condition of the centers of defeat available visual, to ultrasonography or radiological control. Assessment of efficiency of treatment includes also monitoring of indicators of a gemogramma, blood biochemistry, including definition of serumal concentration of markers of functional activity of mast cells; size discrimination of a spleen and liver; assessment of a condition of bone and joint system (densitometry, MRT, a X-ray analysis of bones of 1 times in 1-2 years).
