噬血细胞性淋巴组织细胞增生症47例

目的探讨噬血细胞性淋巴组织细胞增生症(HLH)的临床特点及预后,初步评价早期化疗疗效。方法除诊断所需检测项目外,患儿均常规行细胞及体液免疫功能检测、头部CT或MRI检测,并定期监测上述指标以评估疗效。患者化疗前均常规送检脑脊液生化及常规分类及爱泼斯坦病毒(EBV)、巨细胞病毒(CMV)、单纯疱疹病毒(HSV)、柯萨奇病毒(Cox-V)、埃可病毒(ECHOV)抗体检测。结果1.HLH48例患儿接受化疗,1例死亡,余47例均临床有效,8例因治疗过程中再次出现活动性病变放弃治疗,1例于化疗1.5年后病情再次恶化。25例进入停药观察期,其中3例复发。3年预期生存率为78%。2.疗效差12例中11例(92%)为EBV相关HLH。3.在所有监测指标中脾脏大小变化及铁蛋白可早期反映疾病转归,MRI对脑实质受累的检出率明显高于CT,二者具有非常显著差异(P=0.006)。结论1.化疗过程中应定期监测脾脏大小及血清铁蛋白。2.HLH患者确诊后应常规行头颅MRI检查,并在治疗不同阶段予以监测。3.EBV感染是预后不良因素之一,前期化疗疗效不满意的EBV-HLH应尽早接受干细胞移植。4.适时化疗可明显改善HLH患儿预后。

Hemophagocytic Lymphohistiocytosis in 47 Children

Objective To discuss the clinical characteristic and prognosis of the hemophagocytic lymphohistiocytosis(HLH).Methods Apart from items needed by the diagnosis,cellular and humoral immune function,brain computer tomography(CT) or magnetic resonance imaging(MRI) were examined routinely.All examinations above mentioned were monitored regularly.In addition,cerebrospinal fluid analysis and antibodies of Epstein-Barr virus(EBV),cytomegalovirus(CMV),herpes simplex virus(HSV),Coxsackie virus(Cox-V),echovirus(ECHOV) were detected.Results 1.Altogether 48 children were treated with immunochemotherapy.One child died 3 weeks after the onset of therapy,while the others were in effect and finished initial treatment whose duration was 8 weeks.There were 8 children gave up treatment at the end of the initial treatment because of reactivation.One of the 48 children had been receiving the continuation therapy more than 1 year because of having no resolution,and became worse 18 months after onset of therapy.Twenty-five of the 48 children were off therapy and alive at latest follow-up.Three of 25 children relapsed within 1 month after stopping all therapy.The 3-year probability of survival was 78%.2.Twelve children didn't have good result(recurrence and death).Eleven of them were EBV-HLH.3.Among all the monitoring items,the size of the spleen and serum ferritin could reflect the developing of disease very quickly.Both were sensitive and specific monitoring items.There was significant difference in detection rate of brain substance involvement between CT and MRI(P=0.006).Conclusions 1.Monitoring spleen size and serum ferritin regularly during the course of chemotherapy are recommended strongly.2.All children shall examine brain MRI at the time of diagnosis and recheck at different therapeutic stages.3.EBV infection is one of the unfavorable prognosis.So this group of patients shall adopt bone marrow transplantation as soon as possible,if the therapeutic result is unsatisfactory after the initial treatment.4.Timely immunochemotherapy can improve the prognosis of HLH patients greatly.