Malignant angioendotheliomatosis presenting as disseminated intravascular coagulopathy.

Disseminated intravascular coagulopathy (DIC) occurred in a patient with hemolytic anemia and anasarca. Skin and muscle biopsy showed intravascular lymphomatosis (malignant angioendotheliomatosis). Combination chemotherapy resulted in resolution of the DIC and anasarca. After an unmaintained 8-month clinical remission, the patient had central nervous system relapse and died. Malignant angioendotheliomatosis is a rare disorder that should be considered among the occult causes of DIC.