论文部分内容阅读
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 and diagnosis and treatment of hemophagocytic syndrome (HPS). Methods One HPS with chronic aplastic anemia (AA) was reported and reviewed in combination with the literature. The results of patients with chronic AA history of more than 20a, the admission course of sustained hyperpyrexia, progressive decline in whole blood cells, hyperferrichemia and lactate dehydrogenase hyperlipidemia, bone marrow showed extremely reduced hyperplasia, hemophagocytic cells easily seen, HPS combined with diagnosis of AA, and with serious infection, stimulated bone marrow hematopoietic, anti-infection and other symptomatic supportive treatment to alleviate the disease. Conclusions HPS is dangerous and has a high mortality. Amalgamation with AA is extremely rare. Hematopoietic function is further reduced and treatment is difficult. Early diagnosis and treatment can improve prognosis and increase survival rate.