A Clinical Chart Review And Discussion Of 8 Cases With Hemophagocytic Syndrome

Objective:This clinical research was to explore the etiology,clinical characteristics,clinical experience of diagnosis and treatment and prognosis of 8 cases with Hemophagocytic Syndrome(HPS),and combined with relevant literature review to understand the occurrence and development of Hemophagocytic Syndrome.Methods:A retrospective study was conducted with 8 HPS in preliminary diagnosis from hematology department of the Jinan Central Hospital of Shandong University between January 2014 and January 2017,the content includes:8 patients with Hemophagocytic Syndrome of general information,household heredity factors,clinical manifestation,laboratory examination,pathological findings,and imaging examination,and treatment options,vesting and other materials,and analysis of the discussion.Results:8 patients with HPS were adults.Male 2,female 6,male:female =0.3:1.Stages can be divided by average age(Youth)20-35 years old 2,male 1,female 1.(Adulthood)35-60 years old 4,male 1,female 3.(Old age)60-80 years old,2 cases were women.Age of onset(25-71 years old),average age(50.71 ± 16.92 years old).All the 8(100%)patients newly diagnosed with fever,7cases(87.5%)with splenomegaly,swollen lymph nodes in 8 cases(100%),1 case(12.5%)of skin rash,jaundice in 1 case(12.5%),pleural effusion in 2 cases(25%),abdominal cavity in 1 case(12.5%),pelvic cavity effusion in 2 cases(25%),pericardial effusion in 1 case(12.5%),confirmed by imaging of pulmonary infectionin 5 cases(62.5%).Two lines or three lines of 6 cases(75%)of blood cells is below normal,three lines are reduced in 2 cases,two lines are reduced in 4 cases.Prolonged activation of prothrombin in 5 cases(62.5%),fibrinogen lower than 1.5g/L 3(37.5%).Alanine transaminase in 3 cases(37.5%),aspartate aminotrans’ferase increased in 4 cases(50%).1 case(12.5%)of elevated bilirubin.Albumin decreased by 8 cases(100%).6 cases(75%)of elevated triglycerides,including 4 cases(50%)more than 3mmol/L.Lactate dehydrogenase 6(75%),L-Alanine transferase increased in 7 cases(87.5%).Elevation of serum ferritin in 7 cases(87.5%),of which 5 cases(62.5%)>2000ng/ml.Detection of EB virus DNA negative in 8 cases(100%).Bone marrow blood cells were positive in 8 cases(100%).Etiological analysis of 1 case(12.5%)severe infection.2 cases(25%)suspicious of lymphocyteproliferative tumors,1 case T-cell Lymphoma,1 case were B-cell Lymphoma.3 cases(37.5%)diagnosed with an autoimmune disease,there were 1 case of undifferentiated connective tissue disease,1 case of Adult Onset Still’s Disease,1 case of Systemic Lupus Erythematosus.2 cases(28.6%)for unknown reasons.8 cases of HPS patients,3 cases of etoposide + dexamethasone based chemotherapy(2 cases combined with cyclosporine).2 cases of resistant bacteria,fungi and viruses,such as symptomatic treatment.1 case of hormone and Immunoglobulin treatment of symptomatic support.1 patient(think B-cell Lymphoma),with CVP chemotherapy and symptomatic treatment.Application of dexamethasone in 1 case,cyclosporin A and gammaglobulin treatment.After the initial treatment of 8 patients,4 have been discharged,hospital stay(19-66)days,including 3 cases of patients with autoimmune-related HPS,of unknown aetiology of 1 case of patients with HPS,most commonly 7 months follow-up time.2 patients quit the treatment,automatic discharge lost to follow-up.The remaining 2 cases were dead,and hospitalization for 7 days and 31 days respectively,the mortality rate(25%).Conclusion:1.HPS is a rare and serious disease,if clinical manifestations in patients with persistent fever spleen or lymph nodes,or with a rash,jaundice,such as neuropsychiatric symptoms,blood routine test,abnormal blood clotting protein,iron,and bone marrow examination find hemophagocytic phenomenonso on,the patiert should exclude the HPS as soon as possible,to confirm the diagnosis and treatment in a timely manner.2.Common pathogenic factors of Hemophagocytic Syndrome secondary to malignant tumors,infections and autoimmune diseases,diagnosis of HPS can cover the primary disease clinical manifestations,it can be misdiagnosed or missed diagnosis,so early differential diagnosis is important.3.Hemophagocytic Syndrome complicated with Central Nervous System involvement on clinical rare,difficult to diagnose,related to diagnosis of imaging guide.4.IF the HPS patients have the Hemoglobin,platelets,fibrinogen and serum lactic dehydrogenase and other obvious abnormalities may indicate a poor prognosis in him,should be early intervention,improve the survival rate.