Clinical Analysis Of Hemophagocytic Syndrome In 56 Children

Objective:There are many pathogenic factors for HLH,and viral infection is the most common cause in Asia,mainly seen in epstein-barr virus in herpes virus.EBV-associated Hemophagocytic Lymphohistiocytosis(EBV-HLH)in children has a high incidence frequency and rapid disease progression.The disease is heterogeneous,with diverse clinical manifestations,and early diagnosis is difficult.Because of the particularity of children,if not timely treatment,the death rate is very high.At present,the treatment for HLH mainly consists of HLH-2004,which is divided into induction therapy and maintenance therapy.This paper mainly discusses the clinical manifestations,laboratory examination,therapeutic effect and prognosis of children EBV-HLH,so as to raise the importance of clinicians on this disease.Early diagnosis and early effective treatment will directly determine the survival and prognosis of children.Methods:Selection of Soochow University affiliated children's hospital in August 2011 to August 2018,admitted between clear diagnosis of white blood cells syndrome in patients with clinical data of 56 cases,including 45 cases of EB virus correlation eats blood syndrome(EBV-HLH),the laboratory results and relevant clinical characteristics,treatment and prognosis of outcome etc.Comprehensive analysis was carried out by a retrospective.Results:1.Clinical data of the 56 cases with HLHH,including 26 males(46.4%)and 30 females(53.6%),with a male-female ratio of 0.87 to 1.The median age of onset was 2 years and 11 months(1 day in June-14 years and 7 months).2.Etiology:45 cases(80.3%)were ebv-hlh,5 cases(8.92%)had clear genes related to macrophage syndrome,1 case(1.79%)lymphoma,and 5 cases(8.92%)had no clear etiology.3,the main clinical manifestations of 53 cases(94.64%),fever,liver enlargement 48 cases(85.71%),spleen enlargement of 47 cases(83.92%),superficial lymph node enlargement 38 cases(67.86%),serous cavity effusion in 41 cases(73.21%),digestive system symptoms,including nausea,vomiting,abdominal distention,diarrhea)23 cases(41.07%),nervous system symptoms(including irritability,convulsions,coma)10 cases(17.85%),There were 6 cases of rash(10.7%)and 10 cases of haemorrhagic symptoms(skin mucosal bleeding,nosebleed,gastrointestinal bleeding,hematuria)(17.85%).4.Laboratory examination results:Hemophagocytopenia was found in 50 patients(89.29%)with two or three lines of hemocytopenia,37(66.07%)with bone marrow hemophagocytopenia,38(67.85%)with absolute neutrophil count(ANC)decreased,50(89.29%)with platelets(PLT)decreased,39(69.64%)with fibrinogen(FIB)decreased,and 38(67.86%)with albumin(ALB)decreased.Natural killer cell(NK)decreased in 19 cases(33.92%),serum ferritin(SF)increased in 51 cases(91.07%),alanine transaminase(ALT)increased in 46 cases(82.14%),aspartate aminotransferase(AST)increased in 44 cases(78.57%).Lactate dehydrogenase(LDH)increased in 38 cases(67.86%)and triglyceride(TG)increased in 39 cases(69.64%).5.Among the 56 cases,41 of them adopted the h1h-2004 regimen:dexamethasone(Dex)+etoposide(vp-16)+cyclosporine A(CsA),among which 23 patients received intravenous injection of human immunoglobulin(IVIG).Dex+vp-16 regimen was used in 11 patients,among which IVIG8 cases were added.Two patients were treated with Dex alone,one of whom was treated with IVIG.Two children were treated with Dex+CsA.After treatment,11 cases died due to disease progression and 45 cases were discharged after remission.After treatment,37 of 45 ebv-hlh patients survived and 8 died.Among them,24 patients were treated with IVIG,23 survived and 1 died.Of the 21 patients in the non-ivig group,14 survived and 7 died.Conclusion:The etiology of HLH is complex and the clinical manifestations are often lack of specificity.Typical symptoms and signs are fever,hepatosplenomegaly,hemorrhage,enlarged lymph nodes,and systemic symptoms such as digestive,neurological,and respiratory symptoms.The results of laboratory examination showed hemocytopenia,bone marrow hemophagy,NK cell ratio,decreased GLB and FIB,increased SF,LDH,TG,ALT and AST.HLH is divided into two categories:primary and secondary.Among them,EBV-HLH is the most common secondary HLH in children,and the disease is more dangerous.Therefore,h1h-2004 should be adopted as soon as possible for treatment.Compared with non EBV-HLH,the younger the child is,the higher the incidence rate of EBV-HLH will be.Meanwhile,the higher SF index and the increased LDH in the early onset of EBV-HLH are the adverse factors affecting the prognosis.By comparing the effects of different treatment regimens on the prognosis of children with EBV-HLH,it was concluded that the addition of IVIG in the treatment regimens would be beneficial to improve the prognosis of children with EBV-HLH.