骨髓噬血细胞现象在儿童HLH诊治中意义.docVIP

骨髓噬血细胞现象在儿童HLH诊治中意义【摘要】 目的 探讨骨髓噬血细胞现象在儿童噬血细胞综合征诊治过程中的意义。方法 回顾分析2003年12月至2009年12月在我院儿科住院确诊噬血细胞综合征患儿的骨髓细胞学检查结果,并分析同时期骨髓发现噬血细胞但排除噬血细胞综合征的住院患儿的临床资料。噬血细胞综合征的诊断标准按照HLH-2004方案。结果 ①确诊噬血细胞综合征 61例,其中初次骨髓检查即发现噬血现象的56例(占91.8%),初次骨髓检查未见噬血细胞而在其后3周内2~4次复查发现噬血细胞3例,在疾病过程中始终未见噬血现象2例;②虽然骨髓发现噬血细胞,但排除噬血细胞综合征14例,均经抗感染及对症治疗痊愈。结论 骨髓噬血细胞现象在诊断噬血细胞综合征时已不再是必需条件,没有噬血细胞现象并不能排除噬血细胞综合征的诊断。应根据其他指标,尽早诊断并对患者进行关键性治疗。在治疗过程中注意复查骨髓,对治疗具有指导意义。此外,仅靠骨髓发现噬血现象而诊断噬血细胞综合征也是错误的。 【关键词】噬细胞综合征;噬血细胞性淋巴组织细胞增生症;噬血现象;骨髓 The Role of Hemophagocytosis in Bone Marrow in the Diagnosis and Treatment of Hemophagocytic Lymphohistiocytosis XUE Hong-man,CEN Dan-yang,LIN Chun-yan,et al. Department of Pediatrics,the Second Affiliated Hospital,SUN Yat-Sen University,Guangzhou 510120,China 【Abstract】 Objective To probe into the role of hemophagocytosis in bone marrow in the diagnosis and treatment of hemophagocytic lymphohistiocytosis(HLH). Methods The results of the bone marrow smear of patients confirmed with HLH in pediatric department from December 2003 to December 2009 were retrospectively reviewed,and the clinical data of patients hospitalized in the same period being identified with hemophagocytosis but excluded HLH were analysed.The diagnostic criteria were according to the HLH-2004 protocol.Results ① 61 patients were diagnosed with HLH,among them 56 patients had hemophagocytosis in their initial bone marrow smear,while 3 patients had no hematophagocyte in their first bone marrow aspiration,but after 2~4 times recheckment during the next 2~3 weeks,these three patients had positive bone marrow results.2 patients had no hematophagocyte throughout. ②14 patients excluded HLH revealed hemophagocytosis in their bone marrow,and all these patients recovered after anti-infective and symptomatic treatment.Conclusion Hemophagocytosis in bone marrow is not necessary for the diagnosis ofHLH,confirming that a bone marrow lacking hemophagocytosis does not rule out the diagnosis of HLH,and a negative initial bone marrow aspir