Multiple Hemangiomata With Thrombopenia

Abstract

APPROXIMATELY 60 cases of hemangioma associated with thrombopenia have been reported in the literature since the original description of the syndrome in 1940. The intimate relationship between tumor and platelet count is well known; however, the precise mechanism for the inverse relationship between the size of the tumor and the number of circulating platelets has not been defined. Various forms of therapy have been attempted in this disorder. Contrary to several discouraging reports, the role of corticosteroid treatment is unclear. It is the purpose of this paper to present an instance of a response to steroid therapy which suggests a direct action of this hormone on the hemangioma. In addition, the case itself represents an unusual clinical variant of this syndrome. Report of a Case The patient was a 13-year-old white boy who presented with the syndrome of multiple hemangiomata and severe thrombopenia. He was the product of a full-term