Clinical Features Of Idiopathic Eosinophil Increased Syndrome10Cases Report

OBJECTIVE:To summarize the clinical features of idiopathic hypereosinophilic syndrome in order to improve its diagnosis and treatment.METHODS:The clinical features, treatment and prognosis of10cases of IHES admired to this hospital from January2008to December2013were retrospectively analyzed.RESULTS:①IHES was diagnosed in6male cases and4female cases. The median age was42(23,78) years. The duration of disease was5.5(1,360) months.5cases were misdiagnosed as eczema, enteritis, chronic obstructive pulmonary disease.②Multiple organ dysfunction was showed in clinical features,5cases had fever.6cases had skin lesion.4cases had stomachache and diarrhea.3cases had cough and anhelation.3cases had nervous system symptom.1case had lower limbs arteritis and necrosis.6cases had lymphadenectasis.2cases had ascites,1cases had hydrothorax.③White blood cell increased in9cases. A great number of eosnophils (8.59±2.00×109/L) in blood could be found in all patients.5cases had mild anemia.7cases of erythrocyte sedimentation rate, C-reactive protein were increased and5cases had A/G inversion.④Electrocardiography and the echocardiograms showed abnormal finds in5cases. On the chest CT scan, ground-glass attenuation was identified in3cases, restrictive ventilatory dysfunction was showed in Lung function.⑤Histopathology of interstitial tissue, bone narrow and other involved tissue found a number of nature eosnophils among this patients.⑥(F/P) fusion genes were positive in2cases.⑦Seven cases were treated with glucocorticoid or immunosuppressant. Two cases were treated with casestargeted drugs imatinib. During the follow-up period of nine patients, one case was withdrawn, the other8cases were at stable condition. CONCLUSIONS:①Idiopathic hypereosinophilic syndrome(IHES) is characterized by eosinophilia and infiltration of eosinophilic granulocytes which lead to multisystem damage and may accompanied with fever, weight loss and the signs of inflammation of increased WBC, CRP,ESR etc.②When eosinophilic granulocytes significantly increasing in peripheral blood, bone marrow, or in other tissues, comprehensive examinations ruling out secondary factors or tumors are necessary and may reduce misdiagnosis or missed diagnosis.③F/P fusion gene detection is of significant importance for deeply understanding the classification and treatment of IHES. At present, IHES patients with positive expression F/P fusion gene are included in myeloid or lymphocyte tumors with eosinophilia and abnormity of PDGFR a, PDGFRβ and FGFR1.④First choice drug of the positive F/P fusion gene patients is imatinib. For the negative F/P fusion gene patients, glucocorticoid only or its combination of immunosuppressant is the first choice drug.