成人噬血细胞综合征骨髓噬血细胞形态分析及其临床意义

摘要

Objective To investigate the morphology of marrow hematophagocytes by a retrospective analysis of the marrow smears in 20 cases with hemophagocytic syndrome( HPS) and to probe its clinical significance.Methods The 20 cases were divided into maturity group and infant group by the morphology of the marrow hematophagocytes.the clinical symptoms, signs,laboratory examinations and the mortality rates were compared between the two groups.Results Compared with the characteristics of the infant group, the age of the maturity group was significantly older(P＜0.05) ;the level of platelet and albumin were significantly lower(P＜0.05) and the mortality rate was significantly higher(P＜0.05).There were no statistical differences between two groups in the other items (Hb, WBC, AlT, AST, TB, DB, G, lDH, TG, ferritin, PT, APTT, Fib, fever, hepatolienomegaly).Conclusion The morphology of marrow hematophagocytes is an prognostic factor in HPS.HPS patients in the maturity group have a higher mortality rate, which may be connected with a more serious bleeding tendency and a poorer general condition.%目的 回顾性分析20例临床确诊噬血细胞综合征(HPS)患者骨髓涂片中噬血细胞形态并探讨其临床意义.方法 根据骨髓中噬血细胞形态将20例HPS患者分成成熟组和幼稚组,比较两组患者的临床症状(发热)、体征(年龄、肝脾大)、实验室检查(血常规、肝功能、三酰甘油、乳酸脱氢酶、铁蛋白、凝血功能)及其病死率.结果 成熟组患者较幼稚组年龄大(P<0.05),血小板及清蛋白水平低(P<0.05),病死率高(P<0.05),其余项目如血红蛋白、白细胞、谷丙转氨酶、谷草转氨酶、总胆红素、直接胆红素、球蛋白、乳酸脱氢酶、三酰甘油、铁蛋白、凝血酶原时间、部分活化凝血活酶时间、纤维蛋白原、发热、肝脾大等两组间均无统计学意义.结论 骨髓噬血细胞形态是HPS的预后影响因子,成熟组HPS患者比幼稚组病死率高,这可能与成熟组患者出血倾向的加重和一般状况更差有关.