How to properly and effectively treat various types of histiocytosis
The term "histiocytosis" refers to a number of diseases that are characterized by the proliferation of histiocytes. Depending on the organ that has undergone a granulomatous lesion, there is a histiocytosis of bones, lungs, skin, etc.
The etiology of the disease is not defined. The onset of pathological changes appears as the infiltration of eosinophilic granulocytes and the progressive proliferation of histiocytes. The final phase is fibrosis with insignificant cellular infiltration. In the lungs are found fibrosis, granulomatosis, honeycomb rearrangement of varying degrees of severity.
Electron microscopic examination of washings, which are obtained using alveolar lavage, reveals the bodies of X inside the alveolar macrophages and histiocytes.
Types of histiocyte
Various diseases classified as histiocytosis group are identified:
- Leter-Seee disease. Usually occurs in children under three years of age, always lethal without timely treatment. Defeat is exposed to the skin, bones, lymph nodes, liver, spleen. A frequent complication is pneumothorax.
- Hend-Schüler-Christen Syndrome. Disease, usually found in early childhood, but also in adults, even older people. Damaged mainly bones and lungs, less often - other organs. In some cases, there is a triad of bone defects, diabetes insipidus and exophthalmos.
- Eosinophilic granuloma. This disease is most often found in young people in the 20-40-year-old age. Most often the bone system is affected, in 20% of patients pulmonary infiltration is detected, in some cases the disease affects only the lungs. As with the Hend-Schuiller-Crischen syndrome, spontaneous recovery is also possible in this case. Lethal outcome comes from cardiac or respiratory failure.
Methods of treatment
The defeat of the skin, lymphoid tissues, mucous membranes, macrophages are represented by the cells of Langerhans. Treatment of Langerganscellular histiocytosis at the age of two years has usually a good prognosis. Young patients with multiorgan lesions are in an increased risk of death. Of all patients with histiocytosis, a quarter have a good prognosis for treatment. Low risk is determined by the criteria: age from two years. Absence of lesions of the lungs, liver, hematopoietic system, spleen. At the age from two years and defeat of these organs the patient concerns to group of the high risk.
In the treatment of the disease, general supportive therapy, personal hygiene for limiting the damage to the oral cavity, ears, skin is of great importance. Resection and surgical intervention with severe lesions of gum tissue can reduce the damage to the oral cavity, to reduce the damage to the scalp, use selenium-containing shampoos. Locally, glucocorticoids are used in the affected areas.
Many patients show hormone replacement therapy for diabetes insipidus. When systemic manifestations of the disease patients need to be monitored to identify orthopedic, skin, cosmetic disorders, psychological problems, neurotoxicity.
Chemotherapy is used for multiorgan lesions. In this method, the protocols recommended by the Society of Histiocytosis are used, which are divided into groups of risk. With a good response to therapy, practically in any patient, treatment can be stopped.
Radiation therapy, local surgery is indicated for a disease with bone damage - multiple or single bone. If foci are available in non-critical areas, surgical scraping is recommended. At a risk of impaired organ function, complications of orthopedic or cosmetic nature, surgical methods should be avoided.
Radiation therapy is used at the risk of deformation of the skeleton, pathological fractures, loss of vision, destruction of the spine.
Histiocytosis in adults and children with multiorgan lesions with obvious progression is treated by aggressive chemotherapy, with bad response bone marrow transplantation may be necessary.