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What is the treatment for chronic refractory ITP?

Q: My-5-year old son is suffering from chronic ITP since the past 10 months. We have already given him a 2 months course of steroids (Wysolone) and the doctors have also suggested Lasilactone. After this his platelets count were 75,000 but within 10 days the counts fell to a level of 12-15,000. He also has glands (painless) on the right cervical area, for which a biopsy and a Mantoux test had been done. The doctors have also done a bone marrow test for him. He has also been treated with immunoglobulins and also Anti-D. His counts vary between 35,000 - 40,000. Last week he was rushed to an emergency due to some trauma. Just the day before this incident he had an outpatient appointment and his count that day was 13,000 only. On the day of the trauma, his counts were found to be 16,000 and the doctors monitored him for 24 hours and then repeated the counts, which were then 17,000. The doctors asked us to repeat the counts test after 2 days because the trauma was on the forehead. Today when we went for the counts testing, they were only 1,000 and the manual count only 7,000. The doctors did not admit him and asked us to monitor him and bring him back if any bleeding occurred. We have tried everything and are very worried. Please help.

A:Your son is suffering from Chronic ITP. He has partial response to steroid, IVIg and Anti D earlier. This form of ITP is Chronic Refractory ITP. It shall be important to know that whether bone marrow examination was done before starting steroids in presence of Lymph nodes or not. In chronic ITP rule is that you treat bleeding symptoms rather than treat the number of platelets and try to correct them. The treatment options for chronic refractory ITP are - - Splenectomy - Dapsone - Rituximab - Vincristine - Cyclosporine - High dose Dexamethasone - Azoran We delay splenectomy in children if they are not bleeding. Choice of second line drug depends on the doctor.


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