| Part Ⅰ.Clinical features,laboratory features and prognostic analysis of haemophagocytic lymphohistiocytosisObjective: To retrospectively analyse the characteristic clinical manifestations,laboratory findings,treatment options and regression of patients with haemophagocytic lymphohistiocytosis admitted to our hospital,to explore the important laboratory indicators in the pathogenesis of the disease and to assess the factors influencing their prognosis.Methods: In this study,we retrospectively analyzed the clinical case data of 39 patients with HLH admitted to our hospital from October 2018 to February 2022,statistically analyzed the hemoglobin,neutrophil and platelet counts,serum ferritin level,triglyceride level,liver function,spleen size,whether fever,bone marrow phagocytosis phenomenon,NK cell activity,coagulation function,soluble CD25 and other indicators of patients and The patients were divided into improvement and death groups for comparison,and the differences in laboratory indicators between patients with different prognoses were analysed using SPSS 25.0 software.Results:1.The median age of the 39 patients with HLH was 50(14-96)years,25(64.1%)were male and 14(35.9%)were female.2.Statistical analysis of the laboratory tests and clinical manifestations of the 39 patients with HLH: 39 cases(100%)with fever,35 cases(89.7%)with increased serum ferritin,34 cases(87.2%)with thrombocytopenia,25 cases(64.1%)with liver function impairment,24 cases(61.5%)with splenomegaly,21 cases(53.8%)with moderate to severe anemia,18 cases(46.2%)with hypofibrinemia,and 14 cases(46.2%)with neutropenia.46.2%),neutropenia in 14 cases(35.9%)and hypertriglyceridaemia in 13cases(33.3%).The most common cause of morbidity was infection,with EBV infection present in 19 patients(48.7%(19/39)and CMV infection in 2 patients(5.1%(2/39).15 patients died(38.5% mortality)with a median survival time of 6.1 months.3.Analysis of the differences in laboratory tests comparing patients with different prognoses revealed statistically significant differences in the degree of platelet decline(P=0.002),prolonged APTT(P=0.043)and fibrinogen reduction(P=0.007);fever,spleen size,liver function,triglycerides,PT and bone marrow phagocytosis were not statistically significant in relation to disease prognosis(spleen size: P= 0.603,liver function: P=0.792,triglycerides: P=0.485,PT: P=0.163 and bone marrow phagocytosis:P=0.440).Conclusion: Patients with severe platelet reduction,severe fibrinogen reduction and prolonged APTT may suggest a poor prognosis,while fever,spleen size,liver function,triglycerides,PT and bone marrow phagocytosis are not statistically associated with disease prognosis.Part Ⅱ Analysis of CD47/SIRPα expression of haemophagocytic lymphohistiocytosisObjective: To investigate the expression of the new biological marker CD47/SIRPα in haemophagocytic lymphohistiocytosis and to explore its relationship with the occurrence of HLH as well as its prognosis.Methods: Bone marrow and peripheral blood specimens from HLH patients were examined by flow cytometry and compared with normal controls to analyse the expression of CD47/SIRPα in HLH and to explore its role in the development and prognosis of HLH.Results: The expression of CD47 on the surface of erythrocytes and platelets in the peripheral blood of 13 HLH patients and normal controls was analysed by flow cytometry and found that the expression of CD47 on the surface of platelets was reduced in all cases in HLH patients compared to normal controls,which was statistically significant(P=0.001),while the expression of CD47 on the surface of erythrocytes was not statistically significant(P=0.8136).The expression of CD47 and SIRPα in the peripheral blood of 13 patients with HLH was analysed by flow cytometry and compared with normal controls,and the expression of CD47 and SIRPα in each cell subpopulation was not statistically significant(P>0.05).The degree of CD47 expression on the surface of bone marrow erythrocytes and platelets in 6 cases of HLH was analysed by flow cytometry assay and compared with normal controls,and found that the degree of CD47 expression on the surface of bone marrow erythrocytes and platelets in HLH was not statistically significant(P>0.05).The expression of CD47 and SIRPα in each cell subpopulation of the bone marrow blood of the six cases was analyzed by flow cytometry and compared with normal controls,and it was found that the expression of CD47 and SIRPα in each cell subpopulation was not statistically significant(P>0.05).Conclusion : The expression of CD47 on the surface of platelets in peripheral blood cells was reduced in patients with HLH.The expression of CD47 on the surface of bone marrow erythrocytes,leukocytes and platelets and peripheral blood erythrocytes and leukocytes was not statistically different from normal controls,and the expression of CD47/SIRPα on the surface of each cell subpopulation in bone marrow and peripheral blood did not differ between patients with HLH and normal controls. |
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