Juvenile xanthogranuloma (JXG)
The questions below specifically relate to Juvenile xanthogranuloma (JXG).
1. What causes juvenile xanthogranuloma (JXG)?
JXG involves the over-production of a kind of histiocyte called a dendritic cell. What triggers these cells to accumulate is not known.
2. Is there a cure for JXG?
We usually do not use the term “cure” with this disease, although most patients with only skin or soft tissue JXG have spontaneous remission over time without treatment. Children with liver, bone marrow, CNS involvement and masses in the abdominal cavity usually survive with chemotherapy treatment. There is no established period of inactive disease before JXG is considered cured.
3. What are the different therapies/treatments commonly used to treat JXG?
Patients with a single lesion or just a few lesions, as well as children with skin-only JXG often require no therapy. Surgical removal may be required if the mass is causing organ dysfunction. A small percentage of patients with rapidly growing disease may require treatment with chemotherapy or low-dose radiation, however there is no agreed standard. Steroids have been used to treat eye lesions and in some cases low dose radiation has been effective in preventing visual loss.
4. Can an infant be tested at birth for JXG?
A biopsy of the affected tissue, rather than a blood test, is required for diagnosis and would therefore not be appropriate as a routine test unless this disease is suspected.
Please be advised that all the information you read here is not a replacement for the advice you will get from your consultant and their team.
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