Clinical Characteristics Analysis Of 24 Cases With Hemophagocytic Syndrome

Objective:To analyze the clinical and laboratory characteristics of the HPS,analyze its influence on prognosis,at the same time improve recognitiong and diagnostic level of the disease.Methods:Arrange the clinical data(the clinical features,laboratory examination,treatment and outcome)of 24 patients with Shanxi Medical University from October 2012 to October 2016.24 patients were divided into survival group and death group according to the initial treatment outcome.The data was analyzed by SPSS17.0 software,the measurement data using two independent sample t test,count data using chi square test(Fisher exact test).the P value <0.05 was considered statistically significant.Results:1.Male was 41.67%,female was 58.33%,male/female=0.71:1.The age of onset ranged from 15-78 years old,the median age was 39.5 years.2 All of the 24 patients with secondary HPS,13 cases(54.17%)were secondary to infection,of which including Epstein-barr virus infection(10 cases,41.67%),cytomegalovirus infection(2 cases,8.33%),and EB virus associated with cytomegalovirus infection(1 cases,4.17%).4 cases(16.67%)was lymphoma-associated HPS.The cause of the disease was not clear in 7 patients(29.17%).3.Clinical manifestations included fever(95.83%),splenomegaly(83.33%),lymphadenectasis(66.67%),weight loss(54.17%),digestive system symptoms(75%),respiratorysymptoms(45.83%),bleeding(25%),neurologicalsymptoms(45.83%),serouscavityeffusion(33.33%),rash(8.33%).4.laboratoryexaminationresults:21patients(87.5%)hadhematocytopenia.thefollowingindicatorswerealsomarkedlydecreased:fib(87.5%),albumin(87.5%)andproportionofnkcells(66.67%).however,theseindicatorsincreasedsignificantly:tg(58.33%),alt(83.33%),ast(75%),tbil(62.5%),dbil(62.5%),ibil(37.5%),ldh(79.17%)andsf(95.83%).hemophagocyticphenomenonofmarrowoccurredin20patients(83.33%).inaddition,multipletumormarkerswereabnormalin10patients(41.67%).5.10casesweretreatedwiththehlh-2004regimen(dexamethasone+etoposide+csa),3patientswithvp16+methylprednisolone,1patientwithvp16,2patientswithglucocorticoids,1patientwithe-chopregimen,1patientwithcoepregimen,6patientsweregivensymptomatictreatment.aftertreatment,12 patientsdiedduetodiseaseprogression,anddischargedfromhospitalin12cases.6.therewasastatisticallysignificantdifferenceinthepercentageofnkcells(p=0.04),ldh(p=0.032)andferritin(p=0.023)betweenthesurvivalgroupandthedeathgroup.conclusion:The etiology of hpsiscomplicated,themostcommonetiologyofsecondaryhpsaretheinfectionandtumor.themostcommonclinicalmanifestationsincludefever,splenomegaly,digestivesystemsymptoms.laboratoryexaminationshowedthathematocytopenia,decreasedproportionofnkcells,elevatedserumferritin,elevatedldh,hemophagocyticphenomenonofmarrow,andabnormalfunctionindicatorsofliver,somepatientsalsoshowedunusualmultipletumormarkers.atpresent,themaintreatmentplanisthechemotherapyregimencontainingvp16.attheonsetofthedisease,serumferritin,nkcell ratio and LDH were the adverse factors.