호산구증다증이 동반된 맥관부종 환자에서 혈전성 미세혈관병증이 병발한 1예
A Case of Thrombotic Microangiopathy Associated with Angioedema with Eosinophilia
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Episodic angioedema with eosinophilia (EAE) is characterized by recurrent angioedema, fever, weight gain and eosinophilia in peripheral blood. It has been considered as a distinct clinical entity which is different from the idiopathic hypereosinophilic syndrome. In general, EAE has been known to show a good prognosis and have no internal organ involvement. A couple of recent reports show that EAE could be accompanied by serious complications such as thrombotic microangiopathy (TMA). Here, we report a complicated case of EAE which is believed to be associated with TMA for the first time in Korea. A 38-year-old Korean man initially presented with angioedema, fever, eosinophilia and weight gain which were compatible with EAE. After administration of high-dose glucocorticoids, angioedema and eosinophilia improved; however, severe hemolytic anemia caused by TMA developed suddenly on the 12th day after the medication. Therapeutic plasmapheresis was performed which led to the improvement of all his clinical manifestations. He completely recovered from EAE and hemolytic anemia associated with TMA. The patient has been periodically follow up at our outpatient clinic.
Abstract
Episodic angioedema with eosinophilia (EAE) is characterized by recurrent angioedema, fever, weight gain and eosinophilia in peripheral blood. It has been considered as a distinct clinical entity which is different from the idiopathic hypereosinophilic syndrome. In general, EAE has been known to show a good prognosis and have no internal organ involvement. A couple of recent reports show that EAE could be accompanied by serious complications such as thrombotic microangiopathy (TMA). Here, we report a complicated case of EAE which is believed to be associated with TMA for the first time in Korea. A 38-year-old Korean man initially presented with angioedema, fever, eosinophilia and weight gain which were compatible with EAE. After administration of high-dose glucocorticoids, angioedema and eosinophilia improved; however, severe hemolytic anemia caused by TMA developed suddenly on the 12th day after the medication. Therapeutic plasmapheresis was performed which led to the improvement of all his clinical manifestations. He completely recovered from EAE and hemolytic anemia associated with TMA. The patient has been periodically follow up at our outpatient clinic.
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