Treatment

• Chronic disease—supportive care may be satisfactory in mild cases.

• Iron replacement usually required.

• Trial of steroid/androgens/danazol may 5 symptoms and transfusion need.

• ALG/cyclosporin may be indicated for more severe cases as for aplastic anaemia.

• Acute major thromboses should be treated aggressively with urgent thrombolysis and 10 days heparin. Long-term warfarin mandatory. Consider warfarin prophylaxis after any one clotting episode.

• Severe cases <50 years should be considered for sibling allogeneic transplant if they have a donor—consider MUD in <25 age group if no sibling donor.

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