Abstract:
Objective This study aimed to achieve the early diagnosis and active treatment of adult hemophagocytic syndrome (HPS) and investigate the clinical characteristics and prognostic factors of this syndrome.
Methods A single-center retrospective analysis was performed to analyze clinical characteristics, laboratory findings, and survival data.
Results In 58 patients, the most common clinical manifestations were fever (100%) and splenomegaly (89.7%). The most common laboratory parameters were serum ferritin 500 g/L (100%) and peripheral cytopenia in two or more lineages (96.6%). platelet count, fibrinogen, and lactate dehydrogenase in the death group were significantly lower than in the survival group (P=0.000, 0.001, and 0.000). Survival analysis results showed that infections in the rheumatological group exhibited good prognosis the overall survival (OS) time was not reached in 190 d. Patients with unexplained causes had moderate prognosis (OS time was 60 d); tumor-associated HPS patients had poor prognosis (the OS time was only 30 d). Univariate analysis results showed that patients with Fbg < 1.5 g/L, PLT < 40×109/L, and LDH ≥ 2000 U/L also exhibited poor prognosis (P=0.000). Multivariate analysis results showed that PLT < 40 × 109/L was an independent adverse factor (HR=6.472, 95% CI:1.526-26.065, P=0.011).
Conclusion HPS exhibits complex clinical manifestations and varied etiology. Patients with infection and rheumatism-related HPS had good prognosiss compared with those manifesting tumor-associated HPS. Fbg < 1.5 g/L, PLT < 40×109/L, and LDH≥2 000 U/L were the univariate factors that affected the survival time of patients. PLT < 40×109/L is an independent adverse factor. These patients need systemic treatments as early as possible.
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