Pulmonary haemangiomatosis and large haemangiomas endangering vital structures and with a potential association of consumptive coagulopathy are rare but severe diseases in infancy and childhood.
Therapy with
glucocorticoids,
cytostatics and surgical intervention has not proved satisfactory. From experience with
Kaposi's sarcoma, about 20 children with severe forms of haemangiomatous disease have now been treated with
alpha-interferon with promising results. Mechanisms of action are unknown, but these could include a
cytostatic effect, inhibition of the abnormal proliferation of endothelial cells, fibroblasts and smooth muscle cells. Furthermore,
alpha-interferon could inhibit the paracrine and autocrine effects of locally produced
growth factors. Also,
alpha-interferon could promote the production of
prostacyclin, which inhibits platelet aggregation, leading to reduced damage and consumption of platelets. Long term treatment is mandatory. Initial daily doses of 2 to 5 million U/m2 are administered subcutaneously. Side effects in this dosage interval are uncommon in childhood. Future studies are needed to establish exact guidelines for the use of
alpha-interferon in childhood, but the clinical experience hitherto obtained points to the fact that treatment is effective in severe forms of haemangiomas and pulmonary haemangiomatosis.