The low-risk group was over 2 years old, and the hematopoietic system, liver, lung or spleen were not invaded. The risk group is younger than 2 years old, or the above organs have been violated. Because the disease persists, patients often can't cooperate with the treatment according to the strictly designed scheme, so many symptoms of organ involvement may occur (table 137-2). 0 ~ 2 groups of patients, especially patients with single system diseases. It also won't cause death. Some group ⅱ and most group ⅲ (with multiple system diseases) need systemic treatment, but they are generally effective. The disability rate and mortality rate of young patients with multi-system invasion in group ⅳ are as high as 20%. Although recurrence is common, almost all patients with good curative effect can finally stop treatment. In adult patients, they can also show the ups and downs of chronic diseases.
Severe patients should be hospitalized, given the maximum dose of antibiotics to keep the airway unobstructed, nutritional support (including high-energy nutrition), blood products, skin care, physical therapy and necessary medical care. Strict hygiene measures can effectively reduce the damage to the ear canal, skin and gums. Debridement can even remove severely damaged gingival tissue to limit oral lesions. Seborrheic dermatitis of head can be treated with selenium-containing shampoo (twice a week). If the shampoo doesn't work,
After a complete evaluation of local treatment (surgery and radiotherapy), patients with single bone invasion and patients with multiple lesions in some cases can use local treatment. The patient's lesion is superficial, and it can be curetted in accessible and non-dangerous parts. However, excessive orthopedic and functional injury should be avoided during operation. Local radiotherapy (using high-pressure devices) often causes bone deformities, exophthalmos, decreased vision and pathological fractures.
Low-risk children with chemotherapy > 2 years old, accompanied by a single systemic disease, or bone damage in one or more parts, are often given local treatment instead of systemic treatment. However, ineffective treatment or rising erythrocyte sedimentation rate may indicate the existence of serious systemic diseases. Low-risk patients > 2 years old have a variety of systemic diseases, but they have not invaded the blood system, liver, lungs or spleen. Chemotherapy can be given, which often has a lasting effect, but most of them are < 2 years old, as mentioned above.
Patients with poor prognosis should be matched with HLA at the time of diagnosis, and bone marrow transplantation, cyclosporine or experimental immunosuppression or other immunomodulatory treatments should be considered.
For patients with systemic Langerhans cell histiocytosis, chronic disabilities that may be caused by disease and treatment should be monitored, such as cosmetic or functional orthopedic and skin injury and neurotoxicity, as well as mood swings.