Mastocytosis

The mastocytosis is a disease of the blood circulatory system at which massive special cells – mastocytes, labrocytes and fabric basophiles are formed. При мастоцитозе образуются мастоциты, лаброциты и тканевые базофилы These cells impregnate with themselves different internals and skin.

Classification of a mastocytosis

The mastocytosis has two forms – skin and system.

The skin mastocytosis is, as a rule, a mastocytosis at children. At a mastocytosis children have a pigmental urticaria, diffusion or local makulopapulezny rash on skin of brown or salmoncolored color which arises because of accumulation of a large number of small mast cells.

Less often the diffusion skin mastocytosis at which there is an infiltration of skin mast cells without defeats of discrete character meets.

Most often the skin mastocytosis at children appears aged up to two years.

The system form of a mastocytosis often occurs at adults. It is shown in the form of multifocal damages of marrow, at the same time can also affect skin, lymph nodes, a spleen, a liver and digestive tract.

There are several classifications of a system mastocytosis:

• Painless. Has the positive forecast;

• Associated with other disturbances of hematologic character (with myeloproliferative disturbances, a lymphoma, a myelodisplasia);

• Aggressive mastocytosis. It is characterized by serious organ dysfunction;

• Tuchnokletochny leukemia at which in marrow more than 20% of mast cells are observed there are no damages of skin, multiorgan defeats. Has the bad forecast.

Signs and symptoms of a mastocytosis

The skin mastocytosis generally mentions only integuments. At the same time the following symptoms are observed:

• erubescence;

• skin itch;

• reduced pressure;

• periodic increase of heartbeat;

• periodic fervescence.

The system mastocytosis strikes:

• nervous tissue;

• a spleen (it increases in sizes);

• marrow (normal cells in marrow are replaced with mastocytes, the leukosis forms);

• a liver (the liver increases, condensed, and in it fibrous nodes appear);

• a digestive tract (diarrhea and cankers develops);

• bone system (osteoporosis (a softening of bones) and an osteosclerosis is formed (the bone tissue is replaced with connecting), ostealgias appear);

• lymph nodes (increase and emergence of painful feelings).

Diagnosis

For diagnosis of a mastocytosis investigate blood. Presence at blood of a set of mast cells, and also tryptase and a histamine which are products of their life activity testifies to a mastocytosis.

For Мастоцитоз лечится ПУВА-терапией determination of quantity, a look and extent of development of mast cells, the biopsy of skin, marrow and other struck bodies is carried out.

For identification of any disturbances in a spleen or a liver the KG and ultrasonography is carried out. At damages of a digestive tract endoscopy is applied.

Treatment of a mastocytosis

To start treatment of a mastocytosis it is necessary to address the therapist and the hematologist.

Symptomatic therapy which is directed to suppression of a producing by mast cells of biologically active agents is applied to treatment of a mastocytosis:

• Nedokromil of sodium, Ketotifenum – for stabilization of membranes on mast cells;

• Zodak, Tavegil, Suprastinum – as antihistamines.

In the presence of a diffusion system mastocytosis removal of a spleen is shown.

At a severe form of a mastocytosis or at its antiallergic drugs resistance PUVA-therapy is applied. This therapy effectively reduces quantity of rashes. Improvement is observed after 25 sessions of PUVA-therapy on 3-5äª/cm ² on one session.

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