Table II. Supportive therapy in classical WAS and XLT.
| Prophylactic antibiotics | ||
| 1st line | Trimethoprim/sulfamethoxazole | Children <1 year: 30 mg/kg once daily; children >1 year: 450 mg/m2, rounded to nearest dose band; adults: 960 mg orally once daily (based on trimethoprim component) |
| 2nd line | Azithromycin | Children and adults: 10 mg/kg orally once daily for 3 consecutive days every 14 days, or 3 days per week (maximum 500 mg/day) |
| Immunoglobulin replacement therapy | ||
| 1st line | Subcutaneous immunoglobulin | Typically, weekly to achieve total dose of 300–500 mg/kg over 3 weeks |
| 2nd line | Intravenous immunoglobulin | 300-500 mg/kg intravenously once every 3 weeks |
| Eczema | ||
| 1st line | Topical emollient | Apply at least twice daily |
| 2nd line | Topical 1% hydrocortisone | Apply sparingly to the affected area(s) twice daily |
| or | Topical betamethasone valerate (0·1%) | Apply sparingly to the affected area(s) twice daily |
| or | Topical fluocinolone (0·025%) | Apply sparingly to the affected area(s) twice daily (>3 months of age) |
| or | Topical clobetasol (0·05%) | Apply sparingly to the affected area(s) twice daily (>12 years of age) |
| 3rd line | Oral prednisolone | 1 mg/kg/day orally given in 2 divided doses for 2–3 weeks, then taper gradually, maximum 60 mg od |
| or | Topical tacrolimus (0·03%) | Apply sparingly to the affected area(s) once or twice daily (children >2 years; for children >15 years 0·1% can be used) |
| Active bleeding | ||
| 1st line | Platelet transfusion | Children up to 10 kg: 10–15 ml/kg; children >15 kg and adults: 1 pool, repeated according to clinical response |
| +/− | Aminocaproic acid | Children: 100–200 mg/kg orally as a loading dose, followed by 100 mg/kg every 4–6 h; adults: 4–5 g orally as a loading dose, followed by 1 g/h for 8 h (maximum 30 g/day) |
| Minor nose bleeding | Tranexamic acid | Intravenous preparation used topically |
| ITP | ||
| 1st line | Oral prednisolone | Children and adults: 2 mg/kg/day orally for 1–2 weeks then taper gradually, maximum 60 mg/day |
| or | Methylprednisolone | Children and adults: 4 mg/kg/day intravenously for 4 days then taper gradually, maximum 60 mg/day |
| 1st/2nd line* | Intravenous immunoglobulin | Children and adults: 1 g/kg intravenously as a single dose; consult specialist for guidance on subcutaneous dose *Intravenous immunoglobulin and steroids given together as first line in children |
| 3rd line | Rituximab | 375 mg/m2 weekly for 4 weeks |
ITP, idiopathic thrombocytopenia; WAS, Wiskott-Aldrich syndrome; XLT, X-linked thrombocytopenia.