成人嗜血细胞综合征的临床与实验诊断

目的 探讨临床少见病成人嗜血细胞综合征(HPS)的临床诊断及实验室鉴别诊断的方法 .方法 回顾性分析我院2000年至2008年收治的24例HPS患者的临床资料.结果 24例HPS患者中与恶性肿瘤相关的HPS(MAHS)12例(MAHS组),其中10例由骨髓病理结合免疫组化确诊;非MAHS组12例,其中与病毒感染相关的HPS(VAHS)4例,与其他病原体感染相关的HPS(IAHS)4例,与自身免疫相关的HPS(MAS)4例.MAHS组与非MAHS组在发病年龄、发热持续时间、病死率、血清铁蛋白(FER)水平、血乳酸脱氢酶(LDH)及外周血中性粒细胞碱性磷酸酶(NAP)积分比较差异均有统计学意义(P<0.01,P<0.05).MAHS组骨髓细胞形态、病理特征与非MAHS组比较有显著的差别.结论 病原学、免疫学、骨髓细胞学及病理学有助于早期HPS的诊断及分型,并指导临床的针对性治疗.

Clinical and laboratory diagnosis of adult hemophagocytic syndrome

Objective To investigate the clinical and laboratory diagnosis of adult hemophagocytic syn-drome (HPS) . Methods Clinical data of 24 patients with HPS from 2000 to 2008 were retrospectively analyzed. Results Of the 24 HPS cases, 12 had a malignant associated hemophagocytic syndrome (MHAS), and 10 were fi-nally diagnosed by bone marrow immunohistochemist ;Of 12 cases in non-MAHS group,4 were with virus associated hemophagocytic syndrome (VAHS), and 4 were of the other infections, whereas 4 patients diagnosed of immune associated HS (MAS). There were significant difference in onset age, mortality, serum lactate dehydrogenase (LDH) and serum ferritin(FER) and neutrophilic NAP between non-MAHS group and MAHS group(P <0.01 ,P<0.05). In all cases bone marrow biopsy showed significant differences in cytological and pathological features between MAHS group and non-MAHS group. Conclusion Etiology,immunology,and bone marrow cell biopsy and pathology as well contribute to the diagnosis and typing of HPS and will give a guide to the therapy.