| The hypereosinophilic syndromes (HES) encompass a spectrum ofdiseases that have increased blood eosinophils and tissue damage in common.The clinical manifestations are protean and may involve any organ system, butespecially the heart, skin, lung, or nervous system，so its prognosis is alwayspoor. Epidemically speaking, the morbidity of this disease is low, and theclinical cognition is not enough, so misdiagnosis is common at the early phaseof this disease. In order to make the diagnosis, firstly we should eliminate otherdiseases which can responsively or secondarily cause blood eosinophilsincrease. Early diagnosis and effective treatment really play important roles inthe forward prognosis of this disease. We summarize the researchadvancements of HES on its pathogenesis,classification,diagnosis andtreatment. A retrospective study was conducted in our hospital to analyse thediagnosis ideas and treatments of7HES patients in recent8years.Objective:Through thoroughly reading the relevant report about HES at home andabroad, associated with retrospectivly analysising of the diagnosis andtreatment of HES these years, improve the understanding of this disease.Methods:A retrospective study was done about the HES patients who werediagnosed in the first hospital of JiLin university from May2004to January2012. Among those patients,there are6men and1women, ageing from12years old to67years old, on average of31±18.91. Finally compare thoseclinical data with the latest progress in the study of HES to see whether there are differences.Results:(1) In spite of fully history and related examination, we can not find theexact cause of the disease.Those7cases can be classified as IHES.This fits thestandard of classification and definition at home and abroad.(2) Among those7patients, most of them are men, and the average age ofonset is31±18.9years old, basicly fit epidemiology of HES.(3) Excluding secondary cause that lead to eosinophils rising,presence oftissue damage in common, in associated with increased blood eosinophils,fitthe diagnostic criteria of HES. But in those7cases, blood eosinophilsincreasing period chang from4months to six years,which is not identical withthe diagnostic citeria. This possibly due to early intervention which mayshorten natural progression of disease.(4) There are no genetic abnormity about those5patients who take genetictest.We can not draw a conclusion about there pathogenesis.(5) The clinical manifestations of those7patients are protean and mayinvolve heart, lung, digestive system or blood system damage.This fit the reportthat HES may involve any organ system damage.(6)Steroids is a mainstay of treatment for these patients.Cytotoxic orimmunomodulatory therapies can be used for the management ofsteroid-refractory HES or those who can not suffer from the side effect ofsteroids.But the decision of initial dose and dosage reduction is out ofspecifications.Decreasing drug dose may lead to disease relapse.In addition,through the study of1HES patient with serious heart damage,we found thatserious heart damage can not be cured.(7)Effective rate of treatment is obvious during hospitalization.Butprognosis is not sure for lack of follow-up data.Conclusion: (1) The morbidity and agesex composition of HES which are diagnose inour hospital basicly fit the epidemiological information.(2) The clinical manifestations of those7patients are protean and mayinvolve heart, lung, digestive system or blood system damage.This fit the reportthat HES may involve any organ system damage.(3) Those7cases basicaly fit the diagnostic criteria of HES.For thosewhose period of blood eosinophils increasing under6months may due toearly intervention which shorten natural progression of diease.(4) Refer to Treatment, steroids is the priority drug.Cytotoxic orimmunomodulatory therapies can be used for the management ofsteroid-refractory HES or those who can not suffer from the side effect ofsteroids.But the decision of initial dose and dosage reduction is out ofspecifications.(5) Effective rate of treatment is obvious during hospitalization.Butprognosis is not sure for lack of follow-up data. |
PDF Full Text Request