Author/Authors :
AZIZ, MONA Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan , KHAN, SOFIA W. Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan , AMMAR, TOOBA Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan , MAZARI, NAZISH Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan , FAROOQ, HUSSAIN Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan , NAZAR, NAMREEN Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan , ASIF, MOHAMMAD JAVED Sk. Zyed Postgraduate Medical Institute - Department of Haematology, Pakistan
Abstract :
Hyper eosinophilic syndrome (HES) is a subset of idiopathic eosinophilia that fulfils the criteria of a persistent ( 6 months) increase in absolute eosinophil count (AEC) ( 1.5´109l) associated with target organ damage. Recently it was classified into myeloproliferative and lymphoid variants. Present study is aimed to study the clinico morphological features and variants of HES and their response to various therapeutic modalities. In Pakistani population. It is a case series conducted at Haematology department, Shaikh Zayed hospital, Lahore during 4 years from Jan 2005 to Dec 2008. This study included 8 adult patients of HES diagnosed on history, clinical features and elevated absolute eosinophil count (AEC). Seven of 8 patients were of lymphoid-HES and 1 was of myeloproliferative-HES. M: F ratio is 7:1, mean age of presentation was 37 years in lymphoid- HES and 69 years in myeloproliferative –HES. Presenting features were fatigue, weight loss, fever, SOB, paraesthesia and skin rash. Mean AEC in l-HES was 16´109/l and in myeloprolife- rative-HES was 22.7x109/l. Organ damage was seen in cardiovascular, gastrointestinal (GIT), res- piratory and nervous systems. All of the lymphoid-HES responded to steroids. In conclusion, early diagnosis and targeted therapy improve outcome in HES.