CAJ | 학술논문

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.
목적 탐토림상소견병성인기혈세포종합정(HPS)적림상진단급실험실감별진단적방법 .방법 회고성분석아원2000년지2008년수치적24례HPS환자적림상자료.결과 24례HPS환자중여악성종류상관적HPS(MAHS)12례(MAHS조),기중10례유골수병리결합면역조화학진;비MAHS조12례,기중여병독감염상관적HPS(VAHS)4례,여기타병원체감염상관적HPS(IAHS)4례,여자신면역상관적HPS(MAS)4례.MAHS조여비MAHS조재발병년령、발열지속시간、병사솔、혈청철단백(FER)수평、혈유산탈경매(LDH)급외주혈중성립세포감성린산매(NAP)적분비교차이균유통계학의의(P<0.01,P<0.05).MAHS조골수세포형태、병리특정여비MAHS조비교유현저적차별.결론 병원학、면역학、골수세포학급병이학유조우조기HPS적진단급분형,병지도림상적침대성치료.
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.