CAJ | 학술논문

目的提高对噬血细胞综合征(HPS)的认识和诊断水平,探索对该病的有效治疗手段.方法对多中心72例HPS患者的病因、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 72例患者原发病以病毒感染和T细胞淋巴瘤居多.主要临床表现以持续高热(100%)、脾肿大(83.3%)最为多见.发热(100%)、外周血两系及以上血细胞减低(97.2%)、血清可溶性IL-2受体(Scd25)水平升高(93.1%)以及NK细胞活性降低(94.4%)在诊断中的灵敏度较高.实验室检查发现HPS患者组血清糖化铁蛋白百分比为(17.4±16.0)%.明显低于正常对照的(53.6±13.3)%.而血清TNF-α水平为(143.2±64.8) μg/L,明显高于正常对照的(66.9±19.4) μg/L.多数HPS患者存在肝功能损害(83.6%),以AST升高及低白蛋白血症为主.47例经治HPS患者15周总体生存率为46.8%,其中27例氟达拉滨联合大剂量激素治疗HPS患者总体生存率达63.0%.死亡组患者的血小板计数和纤维蛋白原水平明显低于存活组(P＜0.01).结论 HPS可由多种病因所致,临床表现多样.发热、外周血两系及以上血细胞减低、血清Scd25水平升高以及NK细胞活性降低在诊断中的灵敏度较高.糖化铁蛋白百分比、血清TNF-α水平的变化有助于诊断HPS.氟达拉滨联合大剂量激素是有效的治疗方案.血小板计数和纤维蛋白原水平降低是疾病的不良预后因素.
목적 제고대서혈세포종합정(HPS)적인식화진단수평,탐색대해병적유효치료수단.방법 대다중심72례HPS환자적병인、림상표현、실험실검사지표、치료방안급림상전귀진행회고성분석.결과 72례환자원발병이병독감염화T세포림파류거다.주요림상표현이지속고열(100%)、비종대(83.3%)최위다견.발열(100%)、외주혈량계급이상혈세포감저(97.2%)、혈청가용성IL-2수체(Scd25)수평승고(93.1%)이급NK세포활성강저(94.4%)재진단중적령민도교고.실험실검사발현HPS환자조혈청당화철단백백분비위(17.4±16.0)%.명현저우정상대조적(53.6±13.3)%.이혈청TNF-α수평위(143.2±64.8) μg/L,명현고우정상대조적(66.9±19.4) μg/L.다수HPS환자존재간공능손해(83.6%),이AST승고급저백단백혈증위주.47례경치HPS환자15주총체생존솔위46.8%,기중27례불체랍빈연합대제량격소치료HPS환자총체생존솔체63.0%.사망조환자적혈소판계수화섬유단백원수평명현저우존활조(P＜0.01).결론 HPS가유다충병인소치,림상표현다양.발열、외주혈량계급이상혈세포감저、혈청Scd25수평승고이급NK세포활성강저재진단중적령민도교고.당화철단백백분비、혈청TNF-α수평적변화유조우진단HPS.불체랍빈연합대제량격소시유효적치료방안.혈소판계수화섬유단백원수평강저시질병적불량예후인소.
Objective To explore early diagnosis of hemophagocytic syndrome (HPS) and effective treatment. Methods A multicenter retrospective study was carried out to analyze the causes, clinical features , laboratory findings, treatment and clinical outcomes of 72 patients with HPS. Results Among the 72 patients, EBV infection and T lymphoma were the most common initiating diseases. The most common clinical features were persistent fever (100% ) and splenomegaly (83. 3% ). The diagnostic sensitivity was persistent fever (100% ), peripheral cytopenia in two or more lineages (97.2% ) , high concentration of serum soluble CD25 (93.1% ) and low NK cell activity (94. 4% ). The median percentage of serum glycosylated ferritin was significantly lower in patients in HPS group [(17.4 ± 16. 0)%] than in control group [ (53. 6 ± 13.3)%] (P＜0. 01). And the median level of serum TNF-α was significantly higher in patients group [(143.2 ± 64.8) μg/L] than in controls [(66.9 ±19.4) μg/L] (P＜0.01). Hepatic dysfunction was seen in most patients (83.6% ) mainly manifested as elevated liver enzymes and hypoalbuminemia. The 15-week total survival rate was 46. 8% in 47 treated patients, and was 63% in 27 treated with fludarabine in combination with high dose methylprednisolone. The platelet count and fibrinogen level were significantly lower in death group than in survival group. Conclusions The diagnostic sensitivities of presistent fever, peripheral cytopenia in two or more lineages, high concentration of serum soluble CD25 and low NK cell activity are relatively high and lacking hemophagocytosis does not exclude the diagnosis. Low percentage of glycosylated ferritin and high concentration of TNF-α would be helpful to the diagnosis. High dose methylprednisolone combined with fludarabine is an effective therapy. Platelet count and fibrinogen level are poor prognostic factors for HPS.