Mastocytosis - a disease of the circulatory system, which yields a massive special cells - mast cells, mast cells and tissue basophils.
These cells are impregnated with a different internal organs and skin.
Classification of mastocytosis
Mastocytosis has two forms - cutaneous and systemic.
Cutaneous mastocytosis - is usually mastocytosis in children. When mastocytosis in children appears urticaria pigmentosa, diffuse or local maculopapular rash on the skin brown or orange-pink color, which arises from the accumulation of a large number of small fat cells.
Less common diffuse cutaneous mastocytosis, which occurs when the skin mast cells infiltration unbeaten discrete nature.
In most cases cutaneous mastocytosis in children appears before the age of two years.
Systemic mastocytosis often form occurs in adults. It appears in the form of multifocal lesions of bone marrow this can also affect the skin, lymph nodes, spleen, liver and gastrointestinal tract.
There are several classifications of systemic mastocytosis:
• Painless. It has a positive outlook;
• associated with other hematologic disorders character (with myeloproliferative disorders, lymphoma, myelodysplasia);
• Aggressive mastocytosis. Characterized by severe organ dysfunction;
• mast cell leukemia in which the bone marrow is observed in more than 20% mast cells, no damage to the skin, multiorgan failure. It has a poor prognosis.
Signs and symptoms of mastocytosis
Cutaneous mastocytosis generally affects only the skin. At the same time the following symptoms:
• redness of the skin;
• reduced pressure;
• intermittent palpitations;
• intermittent fever.
Systemic mastocytosis is striking:
• nervous tissue;
• spleen (it increases in size);
• bone marrow (the normal cells in the bone marrow mast cells are replaced formed leukemia);
• liver (the liver is increased, compacted, and it appears the fibrous components);
• digestive tract (diarrhea and appears ulcerative lesions);
• skeletal system (formed osteoporosis (softening of bones) and osteosclerosis (bone tissue is replaced by connective), there are pains in the bones);
• lymph nodes (an increase in the appearance of pain).
To diagnose mastocytosis explore krov.O mastocytosis indicates the presence in the blood of a plurality of mast cells and tryptase and histamine, which are the products of their vital functions.
determining the number, type and degree of development of mast cells, a biopsy of the skin, bone marrow, and other organs affected.
To detect any irregularities in the spleen or liver ultrasound and CT performed. When lesions of the digestive tract endoscopy is used.
Treatment of mastocytosis
To start the treatment mastocytosis should consult a physician and hematologist.
For the treatment of mastocytosis apply symptomatic therapy, which is aimed at suppressing the production of mast cells of biologically active substances:
• Nedocromil sodium Ketotifen - to stabilize the membranes of mast cells;
• Zodak, Tavegil, Suprastin - as antihistamines.
In the presence of diffuse systemic mastocytosis shows removal of the spleen.
In severe mastocytosis or its resistance to allergy medications used PUVA therapy. This therapy effectively reduces the number of lesions. The improvement has been observed after 25 sessions of PUVA therapy 3-5Dzh / cm² at a time.