Diffuse hemangiomatosis, coagulopathy and microangiopathic hemolytic anemia

Described here is an autopsy case of a 30-year-old woman with systemic hemangiomatosis accompanied by coagulopathy and microangiopathic hemolytic anemia. She had hepato-splenomegaly, anemia, thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, and fibrinogenopenia. A splenectomy was performed and a diffuse angioma of the spleen was found. Postmortem examination revealed cavernous hemangiomas and hemangioendotheliomatous lesions in the liver, bone marrow, intestine, and lymph nodes. Coagulation studies suggested that exacerbation of coagulopathy occurred due to Liniac (10MV X-ray) irradiation. Our observation raised the possibility that the irradiation might lead to chronic localized consumption coagulopathy, which was confined to the hemangioma, to acute disseminated types of intravascular coagulation.