噬血细胞综合征合并慢性再生障碍性贫血1例报告并文献复习  

Hemophagocytic syndrome complicated with chronic aplastic anemia:a case report and literature review

作　　者：宋敏[1] 王建宁[1] 孟庆奇[1] 侯艳秋[1] 张柳波[1] 包红雨[1] 傅行才[1] 

机构地区：[1]南京医科大学第二附属医院血液科,210011

出　　处：《中国误诊学杂志》2012年第5期1012-1014,共3页Chinese Journal of Misdiagnostics

基　　金：南京医科大学科技发展基金重点项目(2010NJMUZ49)

摘　　要：目的提高对噬血细胞综合征(HPS)的认识及诊治。方法报告1例HPS合并慢性再生障碍性贫血(AA),同时结合文献进行复习。结果患者慢性AA史20a余,此次入院病程中出现持续高热,全血细胞进行性减少,高铁蛋白血症和高乳酸脱氢酶血症,骨髓示增生极度减低,噬血细胞性组织细胞易见,诊断HPS合并AA,并继伴发严重感染,经刺激骨髓造血、加强抗感染等对症支持治疗后病情缓解。结论 HPS病情凶险,病死率高,合并AA极为少见,造血功能进一步减退,治疗困难,早期诊断和相应治疗利于改善预后,提高生存率。Objective To improve the understanding of hemophagocytic syndrome.Methods Report a case of hemophagocytic syndrome in aplastic anemia and review related literature.Results A 54-year-old female was diagnosed as chronic aplastic anemia more than 20 years ago.In this course of hospitalization,she suffered continuing hyperpyrexia and non of conclusive of infection at the beginning with serum ferritin2 000 ng/ml,LDH 2 250 U/L,Hypoproteinemia,serious pancytopenia and the proliferationbone of marrow cell was few awfully,lymphocyte 74%,reticular cell 26% and 6% with phagocytosis.Besides she suffered serious infection and after strengthening the treatment of anti-infection,IVIG,and so on,the clinical symptom was improved.Conclusions The clinical manifestation of hemophagocytic syndrome is hazardous,case fatality rate is high.It is very rare complicated with AA.Its function of hematopoiesis was slack up further.It is difficult to cure.Early diagnosis and treatment are critical to rescue.

关 键 词：组织细胞增多症/并发症 贫血 再生障碍性 病例报告 

分 类 号：R556.5[医药卫生—血液循环系统疾病]