1. causes of mastocytosis
2. Types of mastocytosis
3. symptoms, diagnosis and treatment of mastocytosis
Mastocytosis - is a complex systemic disease, whichdue to the accumulation and proliferation of mast cells in the tissues of the body.The disease (a malignant form is called a mast cell leukemia) was first described in 1869 as chronic urticaria, leaves behind a brownish stain.The term mastocytosis was introduced only in 1953.It is a rare disease, which is much more common in children, and, as a rule, children mastocytosis limited lesion of the skin, while the adult are almost always develops systemic mastocytosis.
mastocytosis most commonly affects the skin of children, but also may affect other organs, such as intestines, stomach, spleen, liver, lymph nodes or bone.More severe cases of mast cell leukemia accompanied by such a severe blood diseases like acute leukemia, chronic neutropenia, or lymphoma.Approximately 90% of patients with mastocytosis, urticaria pigmentosa
etiology of the disease is unknown.Mast cells are distributed in almost all organs and are located in close proximity to the lymphatic and blood vessels, epithelial surfaces and peripheral nerves, which allows them to carry out protective and regulatory functions, as well as participate in a variety of inflammatory reactions.
pathogenesis of the disease based on the release of various mediators of mast cells, including heparin, histamine, leukotrienes, cytokines and inflammation.An extensive body of infiltration by mast cells, leads to violations of its functions.Among the reasons, which are able to launch the selection of mediators, say heavy exercise, the effect of various toxic substances of chemical or organic origin.Also noted in the etiology of the disease mutation of genes that encode receptor tyrosine kinases.There is a theory about the transmission of the disease in an autosomal dominant way, although most of the patients have no family history.
basis of clinical features, are four forms of the disease:
When cutaneous form of the disease mainly affects the skin and highlights the main symptoms such as itchy skin, heart attacks, skin redness, pressure reduction and intermittent fever.
Cutaneous mastocytosis is characterized by five main types of skin lesions:
If systemic mastocytosis various internal organs mast cells infiltrate.At the same time the diseased organs, there are multiple specific disorders - bone pain, induration and enlargement of the liver, diarrhea, ulcerative lesions of the gastrointestinal tract, enlargement of the spleen, the formation of leukemia, swollen lymph nodes, nervous tissue damage.
basis for diagnosis of mastocytosis are the clinical manifestations of the disease and on the detection of skin and tissues of a large number of fat cells.Apply complex laboratory and instrumental methods of investigation - blood tests, skin biopsy of the affected organs and bone marrow, a chromosomal analysis to identify anomalies, X-rays of bones, MRI, ultrasound and CT scans of internal organs, endoscopic methods of diagnosis.
If mastocytosis in children is not accompanied by any systemic symptoms, the treatment he needs, because it has a great tendency to heal itself through adolescence.Diseases are hematologists and physicians, specific treatments for mastocytosis today is not, therefore, apply symptomatic therapy.Treatment of mastocytosis is based largely on the use of antihistamines, which inhibit the production of biologically active substances by mast cells.When complex forms of mastocytosis, and severe disease shows the use of PUVA therapy.Surgery to remove the spleen as required under diffuse systemic mastocytosis.Forecast treat mastocytosis determined form of the disease and the degree of internal organ involvement.
This article is available exclusively in the educational purposes and is not research material or professional medical advice.
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