Idiopathic hypereosinophilic syndrome associated with rapid progression of cardiac, pulmonary and skin infiltration

Idiopathic hypereosinophilic syndrome (IHES) is a rare myeloproliferative disease characterised by multisystem dysfunction and persistent, extreme eosinophilia of unknown cause. Here we present a 42-year-old patient complaining of moderate to severe chest pain and shortness of breath, and typical ischaemic electrocardiography changes were recorded. He was initially suspected of having acute coronary syndrome, however the coronary angiogram excluded coronary abnormalities. Bone marrow biopsy, left ventriculography, transthoracic echocardiography and cardiac magnetic resonance examinations confirmed the diagnosis of IHES and IHES-mediated cardiac involvement. The patient's illness was alleviated during the first hospitalisation, whereas it had rapidly worsened one month after discharge. In addition, simultaneous pulmonary and skin-infiltrating lesions occurred during the second hospitalisation. The patient's condition improved markedly with combined glucocorticoid, hydroxyurea and warfarin therapy, as well as treatment for heart failure. In this report the diagnostic modalities and treatment strategies for IHES are discussed and reviewed.