A Rare Presentation of a Rare Disease: Hypereosinophilic Syndrome Presenting With Acute Coronary and Cerebrovascular Occlusive Disease

Abstract

INTRODUCTION: Hypereosinophilic Syndrome (HES) is defined by the presence of peripheral eosinophilia of 1.5 x 10*9 /L for at least one month resulting in end organ damage in the absence of secondary causes of eosinophilia [1]. The most common organs involved at presentation are skin and lungs. Early cardiovascular and cerebrovascular involvement is rare [2]. CASE PRESENTATION:A 59 year old female with Diabetes Mellitus and hypertension presented with non-ST segment elevation myocardial infarction of the inferior wall. A few days later the patient developed acute right cerebral and cerebellar infarction confirmed by MRI of the brain.She had leukocytosis with an eosinophil count 79 x 10*3/mcL. Tests for parasitic infection, drug allergy, mast cell dyscrasias, and autoimmune process were negative. Bone marrow biopsy showed hypercellular bone marrow with dysplastic eosinophils suggestive of HES. In the absence of skin disease, negative PDGFRA 2 translocation, and normal vitamin B12 levels, a diagnosis of Idiopathic HES was made. She was treated with high dose steroids, hydroxyurea, and leukapheresis followed by imatinib, with no benefit.Over the next week MRI revealed multiple new bilateral infarcts in the cerebellum and cerebrum. Concomitantly she developed STEMI of the inferior wall. Echocardiogram on day three was normal. On days 6, 11, and 23, echocardiogram revealed worsening restrictive cardiomyopathy with infiltrative disease in both ventricles extending from apex to lateral wall and obliterating the left ventricular cavity resulting in cardiogenic shock and pulmonary edema. Rapidly progressing neurologic, as well as cardiac complications, were attributed to HES. Interleukin-5 receptor antibody, Benralizumab, was started without benefit and the patient died.DISCUSSION: In primary HES, eosinophilic expansion occurs due to underlying stem cell, myeloid, or eosinophilic neoplasm. In secondary HES, polyclonal eosinophilic expansion is driven by eosinophilopoietic cytokines. Neurological and cardiovascular involvement is well described but rare and presents usually in the form of parenchymal involvement and not vascular occlusive disease as in our patient [2].CONCLUSIONS: HES syndrome typically presents with features of gastrointestinal, pulmonary, and skin involvement [1]. Neurological involvement, when seen, is in the form of parenchymal involvement and later in disease course. Our patient instead had arterial occlusive disease involving coronary, cerebral, and vertebral basilar system as the primary manifestation of the disease, which is unique. Similarly, cardiac involvement occurs as a restrictive/infiltrative cardiomyopathy not as coronary artery occlusion. A striking feature was the rapid progression of infiltrative disease of the left ventricle leading to near occlusion of the LV cavity resulting in cardiogenic shock and death within 2 weeks.