Thrombotic storm in Kimura disease

We report herein a patient with Kimura disease who experienced life threatening visceral venous thromboses and a clinical course fitting the description of "thrombotic storm". Kimura disease is an indolent chronic inflammatory disease common in Asians and characterized by angiolymphoid proliferation with ample eosinophil infiltration, peripheral blood eosinophilia and elevated serum immunoglobulin E levels. The clinical course of the disease is thought to be benign. Our patient experienced a major thrombotic event with a thorough evaluation revealing no evidence of inherited thrombophilia or acquired conditions associated with visceral venous thromboembolism. Despite persisting eosinophilia and other manifestations of Kimura disease there have been no recurrent thrombotic events with continuous warfarin therapy over a 4-year period. This case highlights the occurrence of hypercoagulability in association with this unusual eosinophilic disorder.