Hemangiomas and
lymphangiomas are two main types of angiomatous disease that occur most commonly in infancy and childhood. Most
hemangiomas resolve spontaneously, but some endanger vital structures such as the lung, as in pulmonary hemangiomatosis, a rare and universally fatal disease. Occasionally, hemangiomatous lesions are associated with
thrombocytopenia, consumptive coagulopathy (
Kasabach-Merritt syndrome), and
microangiopathic hemolytic anemia. In the past, treatment of hemangiomatosis has included
corticosteroids, cytotoxic drugs,
laser therapy, embolization or other surgical approaches,
radiation therapy,
cryotherapy, and supportive measures such as the administration of platelets or
clotting factors. Recently, it has been found that recombinant
interferon alfa is effective in treating pulmonary hemangiomatosis, as well as other variants of hemangiomatous disease such as
hemangioendotheliomas. Possible mechanisms of action for
interferon include inhibiting proliferation of endothelial cells, smooth muscle cells, or fibroblasts that have been stimulated by endothelial cell or
fibroblast growth factors; enhancing the production of endothelial
prostacyclin; or decreasing the production of
collagen. It is also possible that
interferon alfa antagonizes angiogenesis indirectly through its immunostimulatory actions. With the exception of significant hemodynamic changes in some patients during the first 48 to 72 hours of
therapy with
interferon, side effects are relatively mild.