What is dangerous for histiocytosis in children and how is it treated?
Histiocytosis from Langerhans cells is a designated group of different proliferative processes of a macrophage or histiocytic nature. Langerhans dendritic cells are found in most lymphoid and epithelial tissues, but are mostly present in the epidermis. Histiocytosis in children can occur at any age.
There are several types of this disease:
- Histiocytic lymphoma - is rare, differs malignant course;
- Histiocytic proliferates are an absolutely benign course of the disease.
In the middle of these two forms is a small classification group of rare diseases, which is called "histiocytosis from Langerhans cells".These diseases in the past were part of the group of histiocytosis X and were divided into three forms:
- Hend-Schuller-Christian disease;
- Eosinophilic granuloma;
- disease Lögger-Sive.
Now these forms indicate different manifestations of the same disease. According to the classification of our time, histiocytosis Langerhans is detected in one of its three clinical and morphological species:
- In acute disseminated form;
- In the forms of uni-or multifocal;
- Disseminated acute histiocytosis.
Usually, Langerhans' histiocytosis in children occurs up to two years and rarely occurs in adults.
Clinical picture of the disease
The disease in children is manifested in the form of a rash on the skin resembling seborrheic. In addition, most of the patients with histiocytosis develop lymphadenopathy, hepatosplenomegaly, lung lesions, as well as foci of osteolysis in the bones.
Wide infiltration of bone marrow and organs is often accompanied by anemia, inflammation of the middle ear, thrombocytopenia and mastoiditis. In general, this clinical picture can be observed with acute leukemia. If the disease in the child is not treated, then the lethal outcome quickly occurs.
Half the lives of such patients can be extended to five years with intensive chemotherapy. Disease multifocal histiocytosis of Langerhans is characterized by infiltrates of cells in the bone marrow, which lead to a gradual expansion and destruction of surrounding tissues.
In addition to histiocytes, there are also plasma cells, eosinophils, neutrophils, and lymphocytes. Between these cell elements, the number of eosinophils varies from moderate( loose lying cells) to huge( populations in the form of fields).In the process, any bone is affected, but most often the cranial vault, femur, ribs, as well as multi-focal and focal histiocytic infiltrates are determined in the stomach, lungs and skin.
Main symptoms:
- Rash on the scalp;
- Elevated body temperature;
- Rash in the canal of the external ear;
- Inflammation of the middle ear;
- Exacerbations of mastoiditis;
- Inflammatory respiratory tract.
Treatment of the disease in the hospital
In acute disease, glucocorticoids are prescribed, which are combined with cytostatics( chlorbutin, leukeran, vincristine).
Leukeran is administered at 0, 1 mg / kg taken every day inside, taking 2 weeks, then a 2-week break. Vincristine 1,5 mg once a week. Conduct up to 10 cycles of such treatment. The main therapy in all cases is combined with symptomatic( vitamins, hypothiazide, medicines of the posterior lobe of the pituitary gland).Indications for efficacy are thymosin( thymalin) and decaris( levamisole).
Vincristine 1.5 mg, once a week, or a 0.1 mg / kg leukeran every day for 2 weeks, followed by a 2-week break. Such treatment cycles are carried out about 10 times. With Hend-Schüller-Krischen and Taratynov diseases, if internal organs are not affected, less intensive therapy is performed. In any case, the main therapy is combined with the symptomatic. There are indications of the effectiveness of decaris and thymosin.
The diseases of Taratynov and Hend - Schüller - Krischen are more favorable than Abta - Letterer - Siwa diseases, which are detected timely, at the beginning of treatment.