摘要
目的探讨儿童噬血细胞综合征的临床诊断标准及其骨髓细胞学检查结果对于临床诊断的意义。方法回顾分析2008年5月-2011年5月于我院确诊为噬血细胞综合征的22例患儿的病历资料及骨髓细胞学特点。结果 22例患儿均符合国际组织细胞协会HLH-2004方案诊断标准。均有发热、脾大及血清铁蛋白增高,18/22例(82%)伴外周血至少两系减低,15/19例(79%)伴低纤维蛋白原血症,14/20例(70%)伴高三酰甘油血症,14/22例(64%)患儿在初诊时骨髓中找到噬血细胞。结论骨髓涂片见到噬血细胞不是儿童噬血细胞综合征临床诊断的必备条件,初诊时进行骨髓细胞学检查有助于噬血细胞综合征的诊断与鉴别诊断,在治疗过程中复查骨髓有助于指导治疗。
Objective To discuss the clinical diagnostic standard of childhood hemophagocytic syndrome （HPS） and investigate the significance of bone-marrow cytological results to the clinical diagnosis. Methods A total of 22 children with HPS in Tianjin Children＇s Hospital from May 2008 to May 2011 were enrolled in this study. The bone-marrow cytological features obtained in their initial diagnosis, and their clinical data were analyzed retrospectively. HLH-2004 is adopted as the diagnosis standard. Results All patients were presented with fever, splenomegaly and high-level ferritin; they were in line with the diagnosis standard of HLH-2004. Among them, 81.8% of patients （18/22） were combined with peripheral cytopenia in more than two lineages; 78.9% of patients （15/19） had hypofibrinogenemia; 70% of hypertriglyceridemia and 63.6% had hemophagocytosis. Conclusions Hemophagoc patients （14/20） had ytosis of bone marrow is not necessary in the clinical diagnosis of HPS based on HLH-2004 protocol. However, bone-marrow examination in newly diagnosed Moreover, bone-marrow rechec the patients. patients is suggested for the diagnosis and differential diagnosis of HPS. k is helpful for guiding the treatment and evaluating the condition of
出处
《中国小儿血液与肿瘤杂志》
CAS
2013年第3期132-134,共3页
Journal of China Pediatric Blood and Cancer
关键词
噬血细胞淋巴组织细胞增生症
噬血细胞
骨髓
Hemophagocytic syndrome
Hemophagocytic Lymphohistiocytosis
Hemophagocytosis
bone marrow
