Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature
Idiopathic hypereosinophilic syndrome (HES) is an uncommon disorder which usually presents with prolonged and significant primary eosinophilia with end-organ dysfunction. Damaging proteins released by the eosinophilic granules are responsible for the tissues and organ system damage. Here we repo...
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Main Authors: | , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Pusat Perubatan Universiti Kebangsaan Malaysia
2018
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Online Access: | http://journalarticle.ukm.my/20624/1/23_ms0236_pdf_19378.pdf http://journalarticle.ukm.my/20624/ https://www.medicineandhealthukm.com/toc/13/2 |
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Summary: | Idiopathic hypereosinophilic syndrome (HES) is an uncommon disorder which
usually presents with prolonged and significant primary eosinophilia with end-organ dysfunction. Damaging proteins released by the eosinophilic granules are
responsible for the tissues and organ system damage. Here we report two cases
of idiopathic HES. Both the patients were young lady presented with high grade
fever and concomitant symptoms. Laboratory findings showed leucocytosis with
predominant neutrophilia and marked eosinophilia. A diagnosis of idiopathic HES
was made after excluding secondary causes of eosinophilia. However, the first
patient was complicated with multiple venous thrombosis and intravenous heparin
was started which was later changed to subcutaneous low molecular weight
heparin (LMWH). The patient developed pleural effusion and consolidation.
Intravenous Tazoscin, tablet Prednisolone and tablet Hydroxyurea was started
and the patient responded well. Despite treatment, two weeks later, suddenly the
patient collapsed and unfortunately succumbed. On the other hand, the second
patient was complicated with fever, thrombocytopenia, haemolytic anaemia, acute
renal failure and neurological deficit which were part and parcel of thrombotic
thrombocytopenic purpura (TTP). Plasma exchange was commenced and patient’s
condition had slowly improved. Nevertheless, the hypoxia which she sustained
during the multiple episodes of fits had resulted in permanent brain injury and thus
requiring a tracheostomy for prolonged ventilatory support. Currently, there is no
cure for HES. The main aim of treatment is to minimise the tissue damage caused
by the hypereosinophilia. Early diagnosis and intervention are therefore crucial in
preventing the spread of the disease and the end-organ damage. |
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