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1、11例淋巴瘤相关噬血细胞综合征的临床特点及疗效分析李旭东王东宁何易胡元王文文黄仁魏*（中山大学附属第三医院血液科，广东，广州，510630）【摘要】目的：分析11例淋巴瘤相关噬血细胞综合征（lymphoma-associatedhemophagocyticsyndrome,LAHS)的临床特点并观察临床疗效。方法：2009年3月-2013年10月在我院血液科诊断的11例LAHS患者，分析其临床和实验室检查特点并观察临床疗效。结果：11例患者均有高热、高铁蛋白血症及骨髓中出现噬血现象，10例有全血细胞减少及高甘油三酯血症，9例有脾大及低纤维蛋白原血症。11例患者中2例经化疗
2、后接受异基因造血干细胞移植术，已存活45个月及37个月；1例接受8疗程化疗者已存活15个月；1例化疗后行自体造血干细胞移植术，术后11个月死于感染；6例仅接受化疗者均死亡，生存期4-14个月；另1例未接受治疗即死亡。结论：LAHS具有起病急、病情重、高热、脾大、血细胞减少、噬血现象、高血清铁蛋白及高甘油三酯的临床特点，该类患者诊断困难，化疗疗效差，异基因造血干细胞移植可能是最有效的治疗方法。【关键词】噬血细胞综合征；淋巴瘤；临床特点；疗效Clinicalcharacteristicsandtherapeuticeffectof11casesoflymphoma-assoc
3、iatedhemophagocyticsyndromeLiXudong,WangDongning,HeYi,HuYuan,WangWenwen,HuangRenwei*(DepartmentofHematology,TheThirdAffiliatedHospital,SunYat-senUniversity,Guangzhou,510630,China)[ABSTRACT]Objective:Toobservetheclinicalcharacteristicsandtherapeuticeffectof11casesoflymphoma-associatedhemop
4、hagocyticsyndrome(LAHS).Methods:Theclinicalandlaboratorycharacteristicswereanalysisedandtherapeuticeffectwasobservedin11casesofLAHSbetweenMarch2009andOctober2013.Results:High-gradefever,highserumferritinlevelandhemophagocytosiswereobservedin11cases,cytopeniaandhypertriglyceridemiain10case
5、s,splenomegalyandhypofibrinogenemiain9cases.2patientshavesurvivedseparatelyfor45and37monthsafterchemotherapyandallogeneicperipheralbloodstemcelltransplantation(allo-PBSCT),and1patienthassurvivedfor15monthsafter8cyclesofchemotherapy.1casediedofinfection15monthsafterchemotherapyandautologou
6、speripheralbloodstemcelltransplantation(auto-PBSCT).6caseswhoreceivedchemotherapyonlywerealldeadafter4-14months,and1casediedsoonwithouttherapy.Conclusions:ClinicalcharacteristicsofLAHSareurgentandseriouscondition,high-gradefever,hypertriglyceridemia,cytopenia,hemophagocytosis,highserumfer
7、ritinlevelandhypertriglyceridemia.Allo-PBSCTmaybethemosteffectivetherapyforthesepatientswhilethetherapeuticeffectofchemotherapyispoor.[KeyWords]:hemophagocyticsyndrome,lymphoma,clinicalcharacteristic,therapeuticeffectCorrespondingauthor:HuangRenwei,TEL:020-85252227,
