噬血细胞综合征肺泡出血死亡2例临床分析

Clinical analysis of two death cases of alveolar hemorrhage with hemophagocytic syndrome

目的探讨噬血细胞综合征(HPS)并发肺泡出血的临床表现与诊断。方法对2009年我科收治的2例因肺泡出血死亡的HPS临床表现和实验室检查进行回顾性分析。结果 2例HPS患者在疾病后期均出现严重喘憋,经影像学表现及气管插管后于气道内吸出大量血性分泌物确诊为弥漫性肺泡出血,实验室检查示纤维蛋白原及血小板顽固性减低,最终死亡。结论由低纤维蛋白血症及低血小板血症引起的凝血功能障碍所致肺泡出血是HPS的致死原因之一。注重影像学检查及监测凝血功能有助于及时诊断。早期补充纤维蛋白原、新鲜冰冻血浆及血小板可能有助于改善预后。

Objective To study the clinical manifestations and diagnosis of alveolar hemorrhage in patients with hemophagocytic syndrome.Methods The data of clinical manifestations and laboratory findings of 2 death patients of alveolar hemorrhage with hemophagocytic syndrome were retrospectively reviewed and analyzed.Results These 2 cases of HPS were diagnosed according to their imaging findings and large amount of hemorrhagic secretion sucked from air passage intubation for severe dyspnea at the later stage of HPS,and laboratory findings revealed persistent thrombocytopenia and hypofibrinogenemia,and eventually caused their death.Conclusion Alveolar hemorrhage due to severe coagulation disorders caused by thrombocytopenia and hypofibrinogenemia is one of the main causes for death of patients with HPS.It should be emphasized that imaging examination and monitoring function of blood coagulation may contribute to its timely diagnosis.Early application of fibrinogen,fresh frozen plasma and platelets can probably improve the prognosis of patients with this kind of disease.