| BackgroundHemophagocytic syndrome(HPS),also known as hemophagocytic lymphohis tocytosis(HLH),is divided into primary HPS and secondary HPS.Secondary HPS is divided into virus infection-related HPS(epstein-barr virus,herpes simp lex virus,cytomegalovirus and after vaccination),malignant tumor-related HPS(lymphoma,leukemia,Germ cell tumors and other solid tumors),other infectio n-related HPS(mycoplasma,bacteria,protozoa),immunodeficiency related HPS including chronic granulomatous disease(CGD)and hematopoietic Stem cell tra nsplantation(HSCT).Varicella zoster virus associated hemophagocytic syndrome(VZV-HPS)is a type of virus-associated hemophagocytic syndrome(VAHS),w hich is induced by the primary infection or reactivation of the varicella-zoster virus.The disease,which is difficult to be diagnosed on early stages,has a ra pid onset,complicated clinical manifestations,dangerous condition,and high fat ality rate.At present,there are very few global case reports and clinical charac teristics analysis.ObjectiveThis paper aims to summarize the specificity of VZV-HPS by clinical manifestations,changes in laboratory indicators,treatment plans and prognosis,so as to improve clinicians' early diagnosis,treatment plan selection and prognosis judgment of this disease,improve diagnosis rate,cure rate and reduce mortality rate.MethodsIn this study,a case report was made on the medical records of a patient with varicella-zoster virus-associated hemophagocytic syndrome admitted to the Department of Dermatology,Qilu Hospital of Shandong University in December 2016.This paper retrieved and screened the literature related to varicella-zoster virus-associated hemophagocytic syndrome through CNKI,Wanfang database,Baidu Academic,and PUBMED database,extracted each patient's data in the literature,and sorted and analyzed it,including age,gender,underlying disease,before medication history,family history,clinical symptoms,laboratory indicators,imaging inspection result,treatment time,treatment plan and prognosis,etc..The general situation,specific manifestations,changes of laboratory indicators,treatment plan and disease outcome of VZV-HPS were summarized.Results??Case reportA patient with varicella-zoster virus-related hemophagocytic syndrome was admitted to the Department of Dermatology,Qilu Hospital of Shandong University in December 2016.The clinical data are as follows:a 15-year-old male with a history of mixed allergic purpura for more than 20 days and systemic use of high-dose glucocorticoids More than 20 days history of hormones.He suddenly developed low back pain,epileptic convulsions,high fever,blisters all over the body,pancytopenia,abnormal liver function,and abnormal blood coagulation.He was eventually diagnosed with varicella-zoster virus-related hemophagocytic syndrome.??Literature review and clinical characteristics analysisA total of 17 patients with VZV-HPS from January 1995 to June 2020 were included through literature search.Combined with a patient with varicella-zoster virus-related hemophagocytic syndrome in our hospital,this study involves a total of 18 cases.This paper extracts the medical records of each patient for integrated analysis.1.Gender and age:Among them,14 were male patients and 4 were female patients.The ratio of male to female was 3.5:1.The minimum age is 3 years old.The maximum age is 101 years old.There were 12 cases of patients under 17 years of age,4 cases of 18-65 years of age,and 2 cases of older than 65 years of age.2.Basic diseases,drug history before onset and family history:8 patients(44.4%)with no basic diseases,and 4 patients(22.2%)with stem cell transplantation or solid organ transplantation.There were 11 cases(61.1%)with no drug history before onset,3 cases(16.7%)with glucocorticoid and immunosuppressive agents before onset,and 4 cases(22.2%)with glucocorticoid before onset.There were 18 cases(100%)without familial HPS.3.Predisposing factors:12 cases were induced hemophagocytic syndrome due to VZV primary infection,accounting for 66.7%of the total;6 cases were induced hemophagocytic syndrome because of VZV reactivation,accounting for 33.3%of the total.4.Clinical symptoms:Fever was the most common in 17 cases(94.4%),and body temperature?38.5? in 13 cases(72.2%),most of which showed recurrent high fever between 39? and 40?.7 cases(38.9%)were epistaxis and ecchyma of lower limbs.7 cases(38.9%)had abdominal pain.Neurological symptoms in 5 patients(27.8%);Low back pain in 2 cases(11.1%);Pharyngeal pain in 2 cases(11.1%);Jaundice in 1 case(5.2%);Blindness in 1 case(5.2%).5.Laboratory indicators:100%of patients had different degrees of blood cell reduction,of which 8 cases(44.4%)had three-line reduction of blood cells,and 5 cases(27.8%)had two-line reduction of blood cells;14 patients(77.8%)had PLT<100×10^12/1;10 patients(55.6%)had Hb<90g/1;12 patients(66.7%)had WBC<4.0×10^9/1 or N#<1.0 × 10^9/1;4 cases(22.2%)had RBC<4.3 × 10^9/1;Thirteen patients(72.2%)had significantly elevated transaminases;13 patients(72.2%)had serum ferritin>500ug/l;11 patients(61.1%)had elevated lactate dehydrogenase;5 patients(27.8%)had triglycerides Ester was higher than 256mg/dl;6 cases(33.3%)had fibrinogen<150mg/dl;only 6 cases(33.3%)underwent soluble cluster of differentiation25(sCD25)or soluble interleukin-2 receptor(sIL-2)examination,and the results suggest a significant increase;Only 3 patients(16.7%)underwent natural killer cell(NK)examination,and the results showed decreased activity;83.3%(15 patients)had hemophagocytic cells in bone marrow or lymph node biopsy.Seven patients(47.4%)were not tested for VZV virus due to typical clinical symptoms of varicella or herpes zoster;6 patients(26.3%)polymerase chain reaction of varicella zoster virus(VZV-PCR)were tested positive in blood,blister fluid or cerebrospinal fluid;Five patients(26.3%)were immunoglobulin M of varicella zoster virus(VZV-PCR)positive in their blood.6.Imaging examination:9 cases(50%)had hepatomegaly;7 cases(38.9%)had splenomegaly;5 cases(27.8%)had lymphadenopathy;4 cases(22.3%)had pulmonary inflammation;1 case(5.6%)suffered from pancreatitis with ascites,candidal esophagitis,atrophic gastritis.7.Treatment plan and treatment cycle:antiviral drugs were used in 16 patients(88.9%),most of whom were treated with acyclovir.11 patients(61.1%)were systematically treated with glucocorticoids,mainly medronasone and dexamethasone.Intravenous immunoglobulin was applied in 10 patients(55.6%).Cyclosporin A was used in 2 patients(11.1%).Two patients(11.1%)were treated with vepesid.The treatment cycle is as short as 5 days and as long as 56 days.8.Prognosis:15 patients(83.3%)had complete relief of hemophagocytic symptoms;2 patients(11.1%)died;1 patient(5.6%)gave up treatment.Conclusion1.The incidence of VZV-HPS in males was higher than that in females,and the onset age was mostly in patients aged 17 or below;Organ transplant recipients,immunocompromised patients and patients with a history of immunosuppressive or glucocorticoid use may be susceptible to VZV-HPS.2.The clinical features of VZV-HPS are complex and varied,and all systems of the body can be involved.Fever is the most common symptom.3.For patients with persistent high fever,progressive hemocytopenia and hepat osplenomegity,the examination of Fer,TG,Fib,NK cell activity,sCD25,cell m orphology should be improved as soon as possible to facilitate early diagnosis.4.Most patients with VZV-HPS have a good prognosis;The vast majority of antiviral drugs,glucocorticoids,gamma globulin can achieve complete clinical remission. |
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