Abstract
Introduction: The objective of this paper is to identify the prognostic risk factors of secondary adult hemophagocytic syndrome (HLH) in hospitalized patients and establish a simple and convenient prognostic scoring system. Method:We reviewed 162 adult patients secondary with HLH treated in Zhejiang Cancer Hospital and the First Affiliated Hospital of Medical College of Zhejiang University from January 2014 to December 2018 were enrolled to form the test group; from January 2019 to February 2021, 162 adult patients in the hospitals constituted the validation group. The HLH prognosis scoring system was constructed according to the risk factors, and the patients were divided into three risk groups: low risk, medium risk, and high risk. The scoring system was verified by Kaplan–Meier method and log rank test survival analysis. The discrimination ability was evaluated according to the receiver operating characteristic (ROC) curve. Results: Univariate and multivariate analysis showed that the independent risk factors for the prognosis of HLH were male sex, activated partial prothrombin time (APTT) greater than 36 s, lactate dehydrogenase (LDH) greater than 1000 U/L, and C-reactive protein (CRP) greater than 100 mg/L. The area under the ROC curve was 0.754 (95% Cl: 0.678–0.829). The patients were divided into a low-risk group (0–1), a medium-risk group (2–4), and a high-risk group (5–6). The 5-year overall survival (OS) rate were 87.5%, 41.8% and 12.8%, respectively (p < 0.001). The area under ROC curve was 0.736 (95% Cl: 0.660–0.813) in the validation group, and the 2-year OS of patients in low-risk, medium-risk and high-risk groups were 88.0%, 45.1% and 16.7%, respectively (p < 0.001). Conclusion:The new prognostic scoring system can accurately predict the prognosis of secondary adult HLH and can further provide basis for the accurate treatment of secondary adult HLH.
Highlights
Hemophagocytic syndrome, known as hemophagocytic lymphohistiocytosis (HLH), is a clinical syndrome of immune overactivation, of lymphocytes and histiocytes, with resultant hypercytokinemia [1]
HLH usually presents as an acute or subacute febrile illness associated with multiple organ involvement; most patients with HLH are acutely ill with multiorgan involvement, cytopenias, liver function abnormalities, and neurologic symptoms
Data collected from each patient included age, sex, presumed etiology, presence or absence of splenomegaly, primary disease and laboratory findings (white blood cell (WBC), absolute neutrophil count (ANC), absolute lymphocyte count (ALC), hemoglobin (HGB), platelet (PLT), globulin (GLB), albumin (ALB), aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin (TB), direct bilirubin (DB), indirect bilirubin (IB), triglycerides (TG), lactate dehydrogenase (LDH), creatinine, urea, prothrombin time (PT), activated partial thromboplastin time (APTT), fibrinogen (Fib), C-reactive protein (CRP), ferritin, human immunodeficiency virus antibody, and percentage of macrophages in the bone marrow)
Summary
Hemophagocytic syndrome, known as hemophagocytic lymphohistiocytosis (HLH), is a clinical syndrome of immune overactivation, of lymphocytes and histiocytes, with resultant hypercytokinemia [1]. HLH disease may be associated with specific genetic and/or environmental causes. Primary HLH is a heritable disease conferred by highly penetrant genetic mutations/variations impacting cytolytic functions, lymphocyte survival, or inflammasome activation. HLH usually presents as an acute or subacute febrile illness associated with multiple organ involvement; most patients with HLH are acutely ill with multiorgan involvement, cytopenias, liver function abnormalities, and neurologic symptoms. It has complex etiology, lack of specificity in clinical manifestations, and high mortality. There are few clinical prediction models for the prognosis of secondary.
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call