Idiopathic hypereosinophilic syndrome (HES) is an uncommon disorder which usually presents with prolonged and significant primary eosinophilia with endorgan dysfunction. Damaging proteins eleased 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.
Idiopathic Hypereosinophilic Syndrome with Unusual Presentation: Two Case Reports And Review of Literature
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Sindrom hipereosinofil idiopatik (HES) boleh mengakibatkan morbiditi dan mortaliti yang ketara. Pesakit biasanya mempunyai bilangan darah putih eosinofil yang tinggi untuk jangka masa yang berpanjangan dan menyebabkan tanda-tanda kerosakan organ dalaman. Protein yang merosakkan yang terhasil oleh granul eosinofilik bertanggungjawab terhadap kerosakan tisu dan sistem organ. Kami laporkan dua kes sindrom hipereosinofil idiopatik dan cabaran luar biasa yang ditimbulkan oleh penyakit ini yang jarang berlaku. Kedua-dua pesakit itu adalah wanita muda yang mengalami demam gred tinggi dan gejala bersamaan. Penemuan makmal menunjukkan sel darah putih periferi yang tinggi dengan neutrofilia dan eosinofilia yang dominan. Diagnosis idiopatik HES dibuat selepas tiada punca sekunder eosinofilia dijumpai. Walau bagaimanapun, keadaan pesakit pertama dirumitkan dengan beberapa trombosis vena. Rawatan heparin intravena dimulakan dan kemudiannya ditukar kepada subkutaneus heparin molekul rendah (LMWH). Pesakit kemudian mengalami efusi pleura. Rawatan diteruskan dengan intravena Tazoscin, tablet Prednisolone dan tablet Hydroxyurea dan pesakit beransur pulih. Namun, dua minggu kemudian, tiba-tiba keadaan pesakit kembali parah dan meninggal dunia. Di sisi lain, pesakit kedua mengalami demam, trombositopenia, anemia hemolitik, kegagalan buah pinggang akut dan defisit neurologi yang merupakan sebahagian daripada Trombotik Trombositopenik Purpura (TTP). Pertukaran plasma telah dimulakan dan keadaan pesakit beransur baik. Walau bagaimanapun, beberapa episod hipoksia yang dialami telah mengakibatkan kecederaan otak kekal yang memerlukan trakeostomi untuk bantuan ventilasi yang berpanjangan. Tujuan utama rawatan HES adalah untuk meminimumkan kerosakan tisu yang boleh disebabkan oleh hypereosinofilia. Oleh itu, diagnosis dan rawatan awal adalah penting untuk mencegah penyebaran penyakit dan kerosakan organ.
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