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Malignant angioendotheliomatosis presenting as disseminated intravascular coagulopathy
1Department of Medicine, Emory University School of Medicine, Atlanta, Georgia.
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.
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.