获得性噬血细胞综合征合并多脏器功能障碍综合征病例分析

Analysis of Cases of Acquired Hemophagocytic Syndrome Complicating Multiple Organ Dysfunction Syndrome

目的:探讨获得性噬血细胞综合征(HPS)合并多脏器功能障碍综合征的临床特点,提高对本病的认识。方法:对10例HPS患者的临床病历资料进行综合分析。结果:10例患者均符合HLH-2004获得性HPS的诊断标准,同时伴有3～6个脏器功能障碍,SOFA评分4～12分,平均8.8±2.2分。10例均伴有NK细胞活性减低和sCD25水平升高,经治疗后存活4例,死亡6例。结论:对发热原因不明、多脏器功能障碍的患者,若血细胞减少、肝脾肿大、血清铁蛋白升高,并发NK细胞活性减低和sCD25水平升高,应警惕噬血细胞综合征。早期诊断和及时治疗能够改善预后。

Objective To investigate the clinical features of acquired hemophagocytic syndrome （HPS） complicating multiple organ dysfunction syndrome （MODS）. Methods： Clinical medical records of 10 eases with HPS were analyzed. Results： All cases met the diagnostic criteria of HLH-2004 acquired HPS and presented with 3 to 6 organs dysfunction, SOFA score was 4-12, the median score was 8. 8 ±2. 2. All eases had low activity of NK cells and high level of sCD25. Four of 10 patients survived and 6 died after treatments. Conclusions： HPS should be alerted when patients have a fever of unknown origin, MODS, low activity of NK ceils and high level of sCD25. Early diagnosis and prompt treatment can improve the prognosis.