Hypereosinophilic syndrome in a patient with cardiac involvement
Location
Auditorium Pond Side
Start Date
26-2-2014 10:30 AM
Abstract
Introduction: Idiopathic hypereosinophilic syndrome (HES) is characterized by eosinophilia without identifiable cause and multi-organ dysfunction, most frequently involving heart, nervous system and lungs. This case is remarkable as less than 40% of patients with HES have cardiac involvement. In addition, a literature review revealed no previous report of HES with cardiac involvement from Pakistan.
Case description: A 48-year old male presented to hospital with history of shortness of breath and fever for one month. Physical examination was unremarkable. Laboratory findings demonstrated WBC count of 22 x 109/Lwith 43% eosinophils. Chest x-ray was unremarkable. Spirometry was negative for obstructive and restrictive lung disease. CT scan was unremarkable for any abnormality in lungs, liver, pancreas and kidneys. Tumor markers were negative. Bone marrow biopsy was remarkable for hypercellularity exhibiting increased eosinophils. Echocardiogrpahy was remarkable for normal left ventricular (LV) systolic function, grade II diastolic dysfunction and echogenic densities in both ventricular apices. Cardiac magnetic resonance revealed endomyocardial fibrosis and LV apical thrombus. He was managed with diagnosis of idiopathic HES. Medical management consisted of steroid, hydroxyurea and imatinib mesylate for eosinophilia, ACE inhibitor for LV diastolic dysfunction and oral anticoagulation for apical thrombi. The Patient was followed for one year. His symptoms improved with no shortness of breath now and WBC count is 8 x 109/L with 0.9% eosinophil. Repeat echocardiography revealed normal LV systolic as well as diastolic function.
Discussion: This case illustrates the potential benefit of above treatment for idiopathic HES and ACE inhibitor for LV diastolic dysfunction. Early institution of these drugs can reduce morbidity and mortality. Research on this topic is recommended.
Keywords: Hypereosinophilic syndrome, cardiac involvement
Hypereosinophilic syndrome in a patient with cardiac involvement
Auditorium Pond Side
Introduction: Idiopathic hypereosinophilic syndrome (HES) is characterized by eosinophilia without identifiable cause and multi-organ dysfunction, most frequently involving heart, nervous system and lungs. This case is remarkable as less than 40% of patients with HES have cardiac involvement. In addition, a literature review revealed no previous report of HES with cardiac involvement from Pakistan.
Case description: A 48-year old male presented to hospital with history of shortness of breath and fever for one month. Physical examination was unremarkable. Laboratory findings demonstrated WBC count of 22 x 109/Lwith 43% eosinophils. Chest x-ray was unremarkable. Spirometry was negative for obstructive and restrictive lung disease. CT scan was unremarkable for any abnormality in lungs, liver, pancreas and kidneys. Tumor markers were negative. Bone marrow biopsy was remarkable for hypercellularity exhibiting increased eosinophils. Echocardiogrpahy was remarkable for normal left ventricular (LV) systolic function, grade II diastolic dysfunction and echogenic densities in both ventricular apices. Cardiac magnetic resonance revealed endomyocardial fibrosis and LV apical thrombus. He was managed with diagnosis of idiopathic HES. Medical management consisted of steroid, hydroxyurea and imatinib mesylate for eosinophilia, ACE inhibitor for LV diastolic dysfunction and oral anticoagulation for apical thrombi. The Patient was followed for one year. His symptoms improved with no shortness of breath now and WBC count is 8 x 109/L with 0.9% eosinophil. Repeat echocardiography revealed normal LV systolic as well as diastolic function.
Discussion: This case illustrates the potential benefit of above treatment for idiopathic HES and ACE inhibitor for LV diastolic dysfunction. Early institution of these drugs can reduce morbidity and mortality. Research on this topic is recommended.
Keywords: Hypereosinophilic syndrome, cardiac involvement