Histiocytosis of Langerhans cells is indicated by a group of various proliferative processes macrophage and histiocytic nature.Dendritic Langerhans cells found in most epithelial and lymphoid tissues, but most are represented in the epidermis.Histiocytosis in children can occur at any age.
There are several types of the disease:
- histiocytic lymphoma - a rare, malignant course is different;
- histiocytic proliferative - absolutely benign course of the disease.
the middle of these two forms of classification is a small group of rare diseases, which is called 'cell histiocytosis of Langerhans cells. "These diseases in the past were part of a group of histiocytosis X, and were divided into three forms:
- disease Henda-Shyullera-Christian;
- Eosinophilic granuloma;
- disease Letgerera-Siwa.
Now these forms represent different manifestations of the same disease.In our time classification gistiotsitozLangergansa detected in one of its three clinical-morphological forms:
- In acute disseminated form;
- The uni-forms or multifocal;
- Acute Disseminated histiocytosis.
Langerhans histiocytosis usually occurs in children up to two years and is rare in adults.
clinical picture of the disease
disease in children manifested as the appearance of a skin rash resembling seborrheic.In addition, a large proportion of patients with histiocytosis develop lymphadenopathy, hepatosplenomegaly, lung disease, as well as osteolysis foci in the bones.
wide infiltration of bone marrow and organs are often accompanied by anemia, inflammation of the middle ear, thrombocytopenia and mastoiditis.In general terms, this clinical picture can be observed in acute leukemia forms.If the child's disease is not treated, it quickly comes death.
half of life of these patients manage to extend to five years with the help of intensive chemotherapy.The disease is characterized by Langerhans cell histiocytosis multifocal infiltrates the cells in the bone marrow, which lead to a gradual expansion and destruction of the surrounding tissues.Furthermore
occur histiocytes and plasma cells, eosinophils, neutrophils, lymphocytes.Between these elements eosinophils cells varies from moderate (lying loosely cells) to large (in the form of a population of fields).In the process, any affected bone, but most of the vault of the skull, femur, ribs, as well as multifocal and focal histiocytic infiltrates defined in the stomach, lungs and skin.
- rash on the scalp;
- Increased body temperature;
- rash in the channel of the outer ear;
- inflammation of the middle ear;
- Exacerbations mastoiditis;
- Inflammatory airway processes.
treatment of disease in
hospital with acute course of the disease, prescribe glucocorticoids, which combine with cytostatics (hlorbutin, leykeran, vincristine).
Leykeran appoint 0, 1 mg / kg every day to take inside the reception 2 weeks, then 2-week break.Vincristine 1.5 mg once a week appointment.Spend up to 10 cycles of such treatment.The main therapy in all cases combined with symptomatic (vitamins, hydrochlorothiazide, posterior pituitary medicines).Indications of efficiency are thymosin (thymalin) and decaris (levamisole).
Vincristine 1.5 mg, single technique in a week or leykeran 0.1 mg / kg each day taken orally as well for 2 weeks followed by a 2 week break.Such treatment cycles carried out about 10.In diseases Henda - Shyullera- Taratynova Christian and, if not struck internal organs, spend less intensive therapy.In any case, the basic treatment is combined with symptomatic.There are indications glamor decaris and thymosin.
Diseases Taratynova and Henda - Shyullera - Christian are more favorable than the disease Abt - Letterer - Siwa, which reveal in due time, at the beginning of treatment.