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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2018
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my-ukm.journal.206242022-11-28T13:14:57Z http://journalarticle.ukm.my/20624/ Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature Suria Abdul Aziz, Hafizah H, Nurasyikin Yusof, Azlin Ithnin, Yousuf R, Azma RZ, Hamidah NH, 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. Pusat Perubatan Universiti Kebangsaan Malaysia 2018-12 Article PeerReviewed application/pdf en http://journalarticle.ukm.my/20624/1/23_ms0236_pdf_19378.pdf Suria Abdul Aziz, and Hafizah H, and Nurasyikin Yusof, and Azlin Ithnin, and Yousuf R, and Azma RZ, and Hamidah NH, (2018) Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature. Medicine & Health, 13 (2). pp. 208-216. ISSN 2289-5728 https://www.medicineandhealthukm.com/toc/13/2 |
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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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Article |
author |
Suria Abdul Aziz, Hafizah H, Nurasyikin Yusof, Azlin Ithnin, Yousuf R, Azma RZ, Hamidah NH, |
spellingShingle |
Suria Abdul Aziz, Hafizah H, Nurasyikin Yusof, Azlin Ithnin, Yousuf R, Azma RZ, Hamidah NH, Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
author_facet |
Suria Abdul Aziz, Hafizah H, Nurasyikin Yusof, Azlin Ithnin, Yousuf R, Azma RZ, Hamidah NH, |
author_sort |
Suria Abdul Aziz, |
title |
Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
title_short |
Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
title_full |
Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
title_fullStr |
Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
title_full_unstemmed |
Idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
title_sort |
idiopathic hypereosinophilic syndrome with unusual presentation : two case reports and review of literature |
publisher |
Pusat Perubatan Universiti Kebangsaan Malaysia |
publishDate |
2018 |
url |
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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1751537486885027840 |
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13.2014675 |