异基因造血干细胞移植后伴噬血现象分析

Analysis on hemophagocytic phenomenon after allogeneic hematopoietic stem cell transplantation

目的分析异基因造血干细胞移植(allo-HSCT)后伴噬血现象的特征。方法选取上海市第一人民医院行allo-HSCT后出现噬血细胞性淋巴组织细胞增多症(HLH)的患者2例,讨论其可能的病因。结果患者一入院诊断为免疫缺陷病,行allo-HSCT 13 d后粒系植入,血红蛋白、血小板始终偏低,凝血功能差,消化道出血,巨细胞病毒性肠炎,肺部感染,骨髓细胞形态学检查可见噬血现象。患者二入院诊断为急性单核细胞性白血病,行allo-HSCT 14 d后粒系植入,行allo-HSCT 42 d后三系降低,发热,铁蛋白高,骨髓细胞形态学检查可见噬血现象,嵌合率进行性降低。结论allo-HSCT后伴噬血现象非常罕见,且进展迅速,预后不良,噬血现象常因感染所致。当行allo-HSCT后骨髓出现噬血现象时,应积极排查感染,检测原发病是否复发,同时应检测患者及其父母相关基因,尽早治疗,争取取得更好的预后。

Objective To analyze the characteristics of hemophagocytic phenomenon after allogeneic hematopoietic stem cell transplantation(allo-HSCT).Methods Two patients with hemophagocytic lymphohistiocytosis(HLH)after allo-HSCT were enrolled from Shanghai General Hospital,and the possible causes were discussed.Results The admission diagnosis of case 1 was immunodeficiency disease,granulocyte implantation was found after allo-HSCT for 13 d,hemoglobin and platelet were low,and coagulation function was poor.Gastrointestinal bleeding,cytomegalovirus enteritis and lung infection appeared,and hemophagocytic cells were visible in bone marrow smear.The admission diagnosis of case 2 was acute monocytic leukemia,granulocyte implantation was found after allo-HSCT for 14 d,and peripheral blood cells continued to decrease after allo-HSCT for 42 d.Fever,high ferritin and hemophagocytic phenomenon were visible,and stem cell transplantation chimeric rate declined progressively.Conclusions The hemophagocytic phenomenon after allo-HSCT is very rare,the progress is rapid,the prognosis is poor,and the hemophagocytic phenomenon is often caused by infection.When hemophagocytic phenomenon occurs after hematopoietic stem cell transplantation,it should actively check infection,determine whether primary disease recurs,detect patient and parent related genes,and seek a early therapy and better prognosis as soon as possible.