Source: Annual Congress 2012 - The good clinical practice: useful case report

Abstract

Hypereosinophilic syndrome (HES) is characterized by blood eosinophilic count 1500 or more for 6 months & eosinophillic infiltrations of multiple organs like heart,bonemarrow,skin, CNS.We report a unique case, rare in a female
Case Report-20 years old female had progressive dyspnoea since 1year. One report with her showed peripheral eosinophilia of 27% Respiratory system revealed signs of left pleural effusion wih fluid eosiophilia Evaluated for this presentation Absolute eosinophillic count was 2750 cells. PFT,ECG were normal.CT thorax revealed RA thrombus & RV infiltrations and cardiac MRI showed typical endomyocardial fibrosis in RV.

Echo showed RA thrombus &apical fibrosis in RV. HES was considered strong possibility.Bone marrow showed 37% eosinophils,no blast cells.

Other causes of eosinophillia were ruled out. Diagnosis of HES was confirmed. BCR-ABL& FIP1L1-PDGFRA-a fusion gene were negative.Patient responded to oral steroids & azothoprime.
Discussion-Multiple organ invovement with eosinophils is an important diagnostic criterion of HES.Malignant transformation is known. Early diagnosis,identification of disease variant are required to prevent fatal outcome.

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Y. Anand, R. Chopra, N. Munshi, N. Wadekar, V. Pandharkar, C. Patel, S. Patil, M. Bhosle (Pune, India). Hyper eosinophilic syndrome in a young female – A rare disorder. Eur Respir J 2012; 40: Suppl. 56, 3507

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