Langerhans cell histiocytosis - Treatment

The time of treatment are taken into account the degree of extension of disease, ie the number of organs affected.
Forms located in bone, skin has a good prognosis with spontaneous remission in most cases.In the first phase is administered corticosteroids. If there is remission of lesions are used in chemotherapy. Nitrogen-mustard in topical applications or local irradiation led to good results.
If symptoms of the disease consists of single bone lesion accompanied by pain living with deformation region, pathological fracture, permanent loss of teeth, deafness by affecting rock rising time will be required Intralesional corticosteroids after cleaning the wound, local low-dose radiation therapy 150 cGy / day for 4 days.
If extensive forms of Langerhans cell histiocytosis applied therapy depends on the number of affected organs and their functional disorders. It is used in a first stage to corticosteroids, the administration of prednisone at a dose of 2mg/kg/zit imp 6 weeks, followed by a course of 1 mg / kg / zit imp of 4 weeks, after which there is a cure discontinuous with prednisone. This regimen is applied in cases of patients with multisystem form of Langerhans cell hitiocitoza, but without multiple organic dysfunction.
If severe forms multiorgan dysfunction - impaired lung, bone marrow, affecting hepatosplenic, skin, bone, systemic chemotherapy is recommended. Intravenous Vinblastine is administered once a week at a dose of 0, 05 mg / kg until the occurrence of leukopenia, it is repeated six times.Another regimen consists of administration of chlorambucil at a dose of 5 mg/m2/day for 8 weeks, the dose is halved later stage.
Apply Desmopressin substitution treatment for diabetes insipidus.Cyclosporine and immunosuppressive effects of selective inhibition imunomodulatoarii cellular immune response and slowing the release of cytokines. Administration of 2-clorodeoxiadenozinei and conducting therapy with monoclonal antibodies are therapeutic options for the near future.