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目的:探讨EB病毒相关性噬血细胞综合征(EBV-HPS)的临床特点、治疗以及预后危险因素。方法:对我院37例EBV-HPS患者的临床表现、实验室检查指标、治疗方案及临床转归进行回顾性分析。结果:发热是最常见的临床表现,实验室检查中乳酸脱氢酶(LDH)、铁蛋白及高密度脂蛋白最敏感。与未成年患者相比,成年患者发病时血小板往往更低,低钠低钾血症发生率也较高,肝脏肿大相对少见。37例患者中完全缓解11例,部分缓解4例,无效18例,总有效率为41%(另外有4例仅给予对症支持治疗)。化疗患者有效率为64.7%,明显高于未化疗患者。患者中位随访时间为34个月,1个月生存率为45%,6个月生存率为31%,12个月生存率为25%。治疗后疾病控制情况及是否应用化疗药物是影响预后的重要因素。在进一步治疗的33例患者中,化疗患者1年总生存率达41.2%,激素及丙种球蛋白患者1年总生存率为10.3%(P=0.03)。白蛋白是影响预后的另一个重要指标,发病时中性粒细胞数量、EBV拷贝数及患者年龄均可能影响预后,而LDH及铁蛋白与预后并无明显相关性。结论:LDH、铁蛋白及高密度脂蛋白是诊断EBV-HPS最敏感的指标,未成年患者及成年患者可能具有不完全相同的临床表现。EBV-HPS预后不佳,一旦确诊需要尽快应用以VP16为主的化疗方案。治疗方案是影响预后的主要因素,LDH及铁蛋白与预后并无明显相关性。
Objective: To investigate the clinical features, treatment and risk factors of EBV-HPS. Methods: The clinical manifestations, laboratory indexes, treatment regimens and clinical outcomes of 37 EBV-HPS patients in our hospital were analyzed retrospectively. Results: Fever was the most common clinical manifestation. Lactate dehydrogenase (LDH), ferritin and HDL were the most sensitive in laboratory tests. Compared with juvenile patients, the incidence of adult patients with platelets are often lower, the incidence of hyponatremia, hypokalemia, liver enlargement is relatively rare. Of the 37 patients, 11 were completely relieved, 4 were partially relieved, and 18 were ineffective with a total effective rate of 41% (another 4 had symptomatic supportive care only). Chemotherapy patients effective rate was 64.7%, significantly higher than non-chemotherapy patients. The median follow-up time was 34 months. The 1-month survival rate was 45%, the 6-month survival rate was 31%, and the 12-month survival rate was 25%. Disease control after treatment and whether chemotherapy drugs are important prognostic factors. Among the 33 patients who underwent further treatment, the one-year overall survival rate was 41.2% in chemotherapy patients and 10.3% in patients with hormones and gamma globulin (P = 0.03). Albumin is another important prognostic indicator, the incidence of neutrophils, EBV copy number and patient age may affect the prognosis, and LDH and ferritin and prognosis was not significantly correlated. Conclusions: LDH, ferritin and high density lipoprotein are the most sensitive indicators in diagnosing EBV-HPS. The clinical manifestations may not be the same in both juvenile and adult patients. EBV-HPS poor prognosis, once diagnosed need to apply VP16-based chemotherapy as soon as possible. Treatment is the main factor affecting the prognosis, LDH and ferritin and prognosis is not significantly correlated.