| BackgroundHemophagocytic syndrome(HPS)is a kind of hyperinflammatory response syndrome caused by abnormal immunomodulation,mainly manifested as abnormal activation and proliferation of the CD8+T lymphocytes and the mononuclear-macrophage system,and the proliferation and the production of a large amount of inflammatory cytokines,thereby causing the tissue organ to be damaged,which lead to tissue and organ injury.Persistent fever,hepatosplenomegaly,hematopenia,and hemophagocytosis in bone marrow,liver,spleen,and lymph node tissues were the main clinical features.HPS is not an independent disease,but a group of clinical syndromes with similar clinical characteristics caused by multiple pathogenic factors.The disease is urgent,the clinical manifestations are diverse,and the experimental results are lack of specificity,which can easily lead to misdiagnosis and missed diagnosis;And the disease condition is complex,the progress is dangerous,if not treated in time,the fatality rate is very high.It is of great significance to improve the understanding of the disease,to identify it as soon as possible and to intervene in advance.ObjectivesThrough retrospective analysis of the primary disease,clinical manifestations,laboratory examination,treatment and prognosis factor of adult secondary HPS patients.The aim is to improve clinicians' understanding of the disease,identify its clinical features,strive for early diagnosis and early treatment,improve its therapeutic effect and improve the prognosis of patients.MethodsAll adult patients diagnosed with HPS in the second affiliated hospital of nanchang university from February 2011 to February 2019 were collected.Their age,gender,clinical manifestations,laboratory indicators,etiology,treatment and prognosis were analyzed to understand their characteristics.Results(1)46 adult patients with secondary HPS,including 29 males and 17 females,had a sex ratio of 1.7 to 1.The median age was 52 years(range,18 to 83 years).Young group(age < 60 years): 27 cases,median survival time 52 days;elderly group(age ? 60 years): 19 cases,median survival time 11 days;the difference was statistically significant(P =0.003).(2)The inducing factors of HPS were lymphoma in 23 cases(50.0%),infection in 16 cases(34.8%),autoimmune diseases in 2 cases(4.3%),unknown causes in 5 cases(10.9%).The median survival time of each group was 17 days,22 days,20 days and 11 days,respectively.There was no significant difference in survival time between groups.(3)Clinical features: fever(100.0%),serous effusion(93.5%)and splenomegaly(89.1%)were the most common signs.The most common laboratory indexes were ferritin(100.0%),albumin(100.0%)and hematopenia(95.7%),LDH,93.3%).There were differences in albumin,TG,LDH,creatine,? 2 microglobulin and D-dimer between the young group and the elderly group,P values were 0.024,0.008,0.004,0.002,0.022,respectively.Compared with the young group,the levels of albumin,TG and LDH were lower,and the levels of creatinine,D-dimer and ? 2microglobulin were higher in the elderly group than in the young group.(4)14 patients received chemotherapy based on etoposide and glucocorticoid,11 patients received glucocorticoid therapy,20 patients received symptomatic support therapy,and one patient received hematopoietic stem cell transplantation(HSCT).The median survival time of patients treated with etoposide regimen was longer than that of other treatments(87 days vs 11 days),and the difference was statistically significant(P < 0.05).(5)Patients treated with VP-16 had a longer median survival time(87 days vs.11 days)than those treated with supportive therapy,and the difference wasstatistically significant(P=0.021).Patients treated with VP-16 had a longer survival time.(6)The results of univariate analysis suggested that GLB < 20g/L(P = 0.045),ALP < 100U/L(P =0.047),PT > 16 s(P=0.040)and age ? 60 years(P = 0.003)were the risk factors for prognosis.The results of multivariate analysis suggested that age ?60 years(HR=2.192,95%CI:1.089-4.411,P=0.028),PT > 16s(HR=0.261,95%CI:0.079-0.869,P=0.029)was an independent risk factor for prognosis.Conclusions(1)Adult secondary HPS is a rare disease with similar incidence between men and women,but the majority of middle-aged and elderly patients are male.(2)Lymphoma is the most common cause of secondary HPS in adults,followed by infection,and unknown causes are associated with autoimmune diseases.(3)The clinical manifestations of adult secondary HPS are complicated and varied.Fever,serous effusion,hepatosplenomegaly,increase of ferritin,decrease of albumin,decrease of blood cells and increase of lactate dehydrogenase are more common.(4)The prognosis of adult secondary HPS is poor.Etoposide therapy can improve the prognosis of patients.(5)When the patient's age ? 60 years old,PT > 16 s may be associated with poor prognosis. |
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