key: cord-0029779-8mf2jpxu authors: nan title: 非EB病毒病原体所致感染相关噬血细胞综合征的临床特征及预后 date: 2022-02-03 journal: Zhonghua Xue Ye Xue Za Zhi DOI: 10.3760/cma.j.issn.0253-2727.2022.02.007 sha: 68886c05ca6d1ae735ef8aff7d46d6c652561bf1 doc_id: 29779 cord_uid: 8mf2jpxu OBJECTIVE: To explore the clinical characteristics and outcomes of patients with non-Epstein-Barr virus (EBV) infection-associated hemophagocytic lymphohistiocytosis (IAHLH). METHODS: Clinical data of 48 patients diagnosed with non-EBV IAHLH in Beijing Friendship Hospital from January 2015 to March 2021 were collected, and the clinical characteristics, treatment, curative effect and prognosis of the patients were analyzed retrospectively. RESULTS: This study included 48 patients, 28 males and 20 females, with a median(range)age of 34.5(2–74)years. Pathogens that cause IAHLH were as follows: virus(16 cases, 33.3%), bacteria(17 cases, 35.4%), parasitic agents(13 cases, 27.1%), and fungi(2 cases, 4.2%). The median time from onset to diagnosis of hemophagocytic syndrome(HLH)was 40(10–160)days. The median(range)time duration from prodrome to the definite diagnosis of IAHLH was 67(23–270)days. The clinical characteristics were fever(48 cases, 100%), splenomegaly(34 cases, 70.8%), cytopenia(38 cases, 79.1%), elevated ferritin(45 cases, 93.8%), elevated fasting triglyceride levels(7 cases, 14.6%), hypofibrinogenemia(17 cases, 35.4%), decrease natural killer cell activity(26 in 44 cases, 59.1%), and elevated sCD25(35 cases, 74.5%). Twenty-five patients(52.1%)had adenopathy. Once a certain pathogen was identified as the causative factor of hemophagocytic lymphohistiocytosis(HLH), cytotoxic agents and glucocorticoids were withdrawn, and specific pathogen-directed treatment was initiated. After treatment, 36 cases(75.0%)achieved complete response, and 14 of 15 patients(93.3%)with parasitic and fungal HLH got a response; however, the response rate of patient with bacterial and viral HLH was only 66.7%(22 of 33 patients). The estimated 5-year overall survival rate was 72.3%(95% CI 50.3%–69.8%). The adverse prognostic factors were total bilirubin over the upper limit of normal(OR=20.0, 95% CI 1.1–378.3, P=0.046)and pathogenic infection not fully controlled(OR=19.9, 95% CI 2.9–134.5, P=0.002). CONCLUSION: Non-EBV IAHLH has a good prognosis. When diagnosed, cytotoxic agents and glucocorticoids should be tapered off, and pathogen-targeted therapy should be critically administered to clear the triggering infection. TG>3.0 mmol/L FBG<1.5 g/L NK 细胞活性减低 a 骨髓噬血现象 sCD25 升高 b 肝功能异常 转氨酶>2ULN TBIL>2ULN 白蛋白<30 g/L ALP>2ULN γ-GGT>2ULN 输血 β2-MG 升高 c Hemophagocytic syndromes--an update [J] Adult haemophagocytic syndrome [J] Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults and adolescents-a life-threatening disease: analysis of 133 cases from a single center[J] Recommendations for the management of hemophagocytic lymphohistiocytosis in adults [J] HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis [J] . Pediatr Blood Cancer Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis [J] Hemophagocytic Lymphohistiocytosis in Children: Pathogenesis and Treatment [J] Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management [J] A clinicopathological analysis of 26 patients with infection-associated haemophagocytic lymphohistiocytosis and the importance of bone marrow phagocytosis for the early initiation of immunomodulatory treatment [J] Clinical Characteristics and Outcomes of 101 Children with Hemophagocytic Lymphohistiocytosis: A Four-Year Single-Center Experience from Egypt [J] Tuberculosisassociated haemophagocytic syndrome[J] A clinicopathological analysis of 26 patients with infection-associated haemophagocytic lymphohistiocytosis and the importance of bone marrow phagocytosis for the early initiation of immunomodulatory treatment [J] Factors associated with tuberculosis-associated haemophagocytic syndrome: a multicentre case-control study[J] Current Updates on Classification, Diagnosis and Treatment of Hemophagocytic Lymphohistiocytosis(HLH) [J] Ruxolitinib for refractory/relapsed hemophagocytic lymphohistiocytosis[J] Clinical outcomes of adults with hemophagocytic lymphohistiocytosis treated with the HLH-04 protocol: a retrospective analysis [J] The protective rather than prothrombotic fibrinogen in COVID-19 and other inflammatory states[J] Leukocyte engagement of fibrin (ogen)via the integrin receptor alphaMbeta2/Mac-1 is critical for host inflammatory response in vivo[J] A retrospective analysis of 56 children with hemophagocytic lymphohistiocytosis[J]