Retrospective analysis of clinics and the prognosis of 58 adult patients with hemophagocytic syndrome in a single center
10.3969/j.issn.1000-8179.20131885
- VernacularTitle:58例成人噬血细胞综合征回顾性临床和预后分析
- Author:
Fei LI
;
Pu LI
;
Rongyan ZHANG
;
Dexiang JI
;
Qian XU
;
Ganping YANG
;
Xianbao HUANG
;
Yanlin WEI
;
Ruibin HUANG
;
Guoan CHEN
- Publication Type:Journal Article
- Keywords:
hemophagocytic syndrome;
clinical manifestations;
laboratory findings;
prognosis
- From:
Chinese Journal of Clinical Oncology
2014;(5):324-327
- CountryChina
- Language:Chinese
-
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 rheu-matological 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.