Table 1 Management options for KHEa
From: Kaposiform hemangioendothelioma: current knowledge and future perspectives
Specific approach | Dosage | Comment |
|---|---|---|
Systemic | ||
 Corticosteroids | Oral prednisolone 2 mg/kg/d or intravenous methylprednisolone 1.6 mg/kg/d | Corticosteroid plus vincristine or corticosteroid plus sirolimus is recommended as the first-line therapy for severe KMP. Corticosteroids should be tapered as soon as medically feasible. |
 Vincristine | Intravenous 0.05 mg/kg once weekly | Vincristine plus aspirin and/or ticlopidine and vincristine plus corticosteroid are recommended for severe KMP. |
 Sirolimus | Oral 0.8 mg/m2/dose twice daily, adjust for a trough level of 8–15 mg/ml | Sirolimus plus corticosteroid is now recommended as the first-line therapy for severe KMP. Lower serum levels (2–5 mg/ml) of sirolimus are recommended for long-term use or toxicities. |
 Ticlopidine | Oral 10 mg/kg/d | Monotherapy is not recommended for KMP. |
 Interferon-α | Subcutaneous injection, 1–3 × 106 U/m2 | Interferon-α is not recommended for patients younger than 1 year old due to its significant neurologic toxicities. |
 Propranolol | Oral 2–3 mg/kg/d | Monotherapy is not recommended for KMP. |
Topical | ||
 Sirolimus gel | 0.1%, twice daily | Used for superficial KHE. |
 Tacrolimus ointment | 0.1%, twice daily | Used for superficial KHE. |
Supportive care treatments | ||
 Fresh frozen plasma or cryoprecipitate | The dosage used is based on the actual situation of the patient (e.g., the severity of hypofibrinogenemia). | Used for active bleeding, platelet count < 30 × 109/L, and/or fibrinogen < 1.0 g/L. |
 Platelets | The dosage used is based on the actual situation of the patient (e.g., the severity of thrombocytopenia). | Platelet transfusion is only recommended for active bleeding or before a surgical procedure when the platelet count is less than 30 × 109/L. |
 Packed red blood cells | The dosage used is based on the actual situation of the patient (e.g., the severity of anemia). | Transfusion of packed red blood cells is recommended for patients who have symptomatic anemia (hemoglobin concentration less than 80 g/L). |
 Active nonintervention | Adjust scheduled visits on the basis of growth of the tumor and/or the development of complications. | Careful observation is recommended for unchanged and uncomplicated KHE. |