Analysis of Prognostic Risk Factors and Establishment of Prognostic Scoring System for Secondary Adult Hemophagocytic Syndrome

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.

Prognostic Factors of Adult Hemophagocytic Lymphohistiocytosis and Clinical Utility of HLH-2004 Diagnostic Criteria and HScore: A Real-World Multicenter Study from Thailand

“…Although ferritin is a non-specific test for HLH diagnosis in Systemic Inflammatory Response Syndrome [37] and hematologic malignancy [38], test results can guide a physician to proceed with more investigations to complete the HLH-2004 criteria and HScore and avoid a delayed diagnosis. Hypoalbuminemia (<2 g/dL), high LDH level (>1,000 U/L), and prolonged PTT (>36 s) were not found to be correlated with poor outcomes in our study [20,34,39,40]. However, a platelet count <20 × 10 9 /L was associated with poor survival in the univariate analysis [20].…”

Elevated Interleukin-10 Levels Are Associated with Low Platelet Count and Poor Prognosis in 90 Adult Patients with Hemophagocytic Lymphohistiocytosis

“…Otrock et al [10] found that only malignancy was an independent risk factor for a poor prognosis. Some singlecenter studies from China discovered other parameters that impacted prognosis in adult HLH including IL-10 ≥129 pg/mL, male, IL-6 ≥18.59 pg/mL, APTT >36 s, LDH >1,000 U/L, and CRP >100 mg/L [11,14,23]. A multicenter study suggested that gender, platelet count, albumin, ALT, and treatment regimens were independent prognostic factors for HLH in adults [24].…”

Secondary hemophagocytic syndrome in an acquired immunodeficiency syndrome and Alpha-thalassemia patient infected with Talaromyces marneffei: A case report and literature review

“…Upon TSM diagnosis, antibiotics were promptly adjusted to Amb. Furthermore, if a male patient presents with an activated partial thromboplastin time greater than 36 s, LDH greater than 1000 U/L, and CRP greater than 100 mg/L, this combination is consistently associated with a poor prognosis for HLH [24] . Our patient has been undergoing follow-up at the outpatient department for infectious diseases for three years post-discharge, and currently, their condition remains effectively controlled without any signs of recurrence.…”