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Histiocytosis
How is it treated?
In time Langerhans Cell Histiocytosis (LCH) may burn itself out, but there may be long-standing problems due to damage caused by the disease process. Not all patients require specific treatment but, when this is indicated, they usually receive corticosteroids (Prednisolone) or cytotoxic drugs.
Haemophagocytic Lymphohistiocytosis (HLH) is often triggered by viral or other infection and improvement generally follows treatment for the precipitating infection and removal of immune suppression, if this is possible. Primary HLH is a genetic disorder which usually presents in very young children with no other known immune abnormality. This group of children usually require drug treatment with corticosteroids, cytotoxic chemotherapy, or the immune suppressive drugs antithymocyte globulin and Cyclosporin. Children with a definite family history (familial HLH) generally receive a bone marrow transplant once the disease is adequately controlled and this is often also necessary for other children with primary HLH who do not have a definite family history.
What are the causes?
| Inheritance patterns and prenatal diagnosis 
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