Clinical Analysis Of86Cases With Adult Hemophagocytic Syndrome

Objective To investigate the etiology，clinical features, survival rate andprognostic risk factors of secondary hemophagocytic syndrome.Methods A retrospective study was performed to analyze the clinical dataof86patients with secondary hemophagocytic syndrome in First AffiliatedHospital of Guangxi Medical University from September2008to September2011.The follow-up was via telephone to confirm the condition of the patients.Data was analyzed with SPSS17.0.Results1. In this study,65patients were male (75.6%) and21patients were female(24.4%). The median age at diagnosis was41years (range:18–80years).2. The etiology was unknown in36cases (41.9%). And28cases (32.6%) wererelated to malignancies, of which Lymphoma-associated HLH waspredominant.19cases (22.1%) were related to infection，of which EBvirus-associated HLH was predominant. Besides,3patients (3.5%) wererelated to autoimmune diseases.3. The main clinical manifestations included prolonged fever(100%),splenomegaly(44.2%), hepatomegaly(41.9%)， lymphadenectasis(46.5%), serous cavity effusions (53.5%) and respiratory symptoms(54.7%). In addition，jaundice and erythra were also common.4. Laboratory features included cytopenia (100%), increased lactate dehydrogenase (80.2%), elevated serum ferritin (86.4%), decreasedpercentation of NK-cell (47.7%), hypofibrinogenemia (38.4%) andhemophagocytosis (90.7%). Besides, hyponatremia, liver dysfunction andhypoalbuminemia were also common.38patients undertook the β2-MG testand β2-MG elevated in all these patients.5. In the study,6patients gave up treatment, and7patients were lost offollow-up. Overall survival at1month,100days and one year were52.1%、27.4%and11.0%respectively in these73patients. The survivalrates at1month,100days and1year were28.9%,18.4%and10.5%in thepatients who accepted the general supporting treatment. And the survivalrates at1month,100days and1year were84.6%,61.5%and30.8%forthose treated with etoposide.6. The result showed that decreased percentation of NK-cell andhemophagocytosis had prognostic value, which was statistically significant(P=0.000,0.014).Conclusion1. The causes of secondary hemophagocytic lymphohistiocytosis (HLH) arevarious, most of which are unknown. The most common defined cause islymphoma, the secondary one is infection, especially EB virus infection.2. The clinical manifestations are various. Prolonged fever and splenomegalyare very common. Besides, serous cavity effusions and respiratorysymptoms are not rare.3. Cytopenia， elevated serum ferritin，increased lactate dehydrogenase andhemophagocytosis are sensitive diagnostic indicators of HLH. EBvirus-associated infection can not be excluded even when repeated tests forEB virus-antibody are negative. If the patients suspected to or diagnosed as HLH, the EBERs in situ hybridization and bone marrow biopsy should beperformed simultaneously to increase the positive rate.4. We conclude that early administration of etoposide is effective in thepatients with HLH.5. HLH has poor prognosis and high mortality, decreased percentation ofNK-cell and hemophagocytosis were the prognostic risk factors of HLH.Therefore, early diagnosis and early treatment are crucial for improving thecure rate of HLH.