溶血指标联合监测在淋巴瘤相关噬血细胞综合征继发Evans综合征疗效评估中的价值OA
Value of combined monitoring of hemolytic reaction indicatorsin efficacy assessment of lymphoma-associated he-mophagocytic lymphohistiocytosis secondary to Evans syndrome:a case report and literature review
目的 探讨溶血指标联合监测在淋巴瘤相关噬血细胞综合征(lymphoma-associated hemophagocytic lym-phohistiocytosis,LA-HLH)继发 Evans 综合征(Evans syndrome,ES)疗效评估中的价值.方法 纳入1 例 LA-HLH 继发 ES 并发生溶血危象的患者,在患者因重度贫血接受必要红细胞输注的前提下,对输血后血红蛋白(hemoglobin,Hb)增幅、网织红细胞百分比(reticulocyte percentage,RET%)、总胆红素(total bilirubin,TBil)、乳酸脱氢酶(lactate dehydrogenase,LDH)及直接抗人球蛋白试验(direct antiglobulin test,DAT)等溶血反应指标进行联合动态监测,结合近 5 年文献,分析上述指标动态变化与免疫抑制治疗应答的关系.结果 在溶血危象期,患者连续 2 次红细胞输注后24 h Hb 增幅均<10 g/L,表现为无效输血;强化免疫抑制治疗后,连续 2 次输注后 Hb 增幅分别达+14 g/L 和+15 g/L,转为有效输血.同期RET%由峰值29.32%降至12.71%,LDH 及TBil 同步改善.将Hb 增幅与RET%动态变化趋势、DAT 结果联合解读,有助于区分免疫性溶血、噬血现象和骨髓衰竭在贫血中的相对权重.结论 在严格把握输血适应证和患者安全的前提下,对溶血指标进行联合动态监测,可为 LA-HLH 继发 ES 的疗效评估提供实时、客观的床旁辅助信息,对优化治疗调整节点具有一定临床价值.
Objective To investigate the value of combined monitoring of hemolytic reaction indicators in the efficacy assessment of lymphomaassociated hemophagocytic lymphohistiocytosis(LAHLH)secondary to Evans syndrome(ES).Methods One patient with LAHLH secondary to ES complicated by hemolytic crisis was enrolled.Under the premise that necessary red blood cell transfusions were administered due to severe anemia,combined dynamic monitoring was performed for the following hemolytic reaction indicators:posttransfusion hemoglobin increment,reticulocyte percentage(RET%),to-tal bilirubin(TBil),lactate dehydrogenase(LDH),and direct antiglobulin test(DAT).Furthermore,based on the litera-ture of the past five years,we analyzed the relationship between the dynamic changes of the these indicators and the response to immunosuppressive therapy.Results During the hemolytic crisis period,the 24hour posttransfusion hemoglobin incre-ments were both<10 g/L after two consecutive red blood cell transfusions,indicating ineffective transfusion.After intensi-fied immunosuppressive therapy,the hemoglobin increments reached+14 g/L and+15 g/L,respectively,after two subse-quent transfusions,converting to effective transfusion.Concurrently,RET%decreased from a peak of 29.32%to 12.71%,and LDH and bilirubin improved synchronously.The combined interpretation of hemoglobin increment,dynamic RET%trends,and DAT results facilitated the distinction of the relative contributions of immune hemolysis,hemophagocytosis,and bone marrow failure to anemia.Conclusion Under the strict premise that transfusion indications are adhered to and patient safety is ensured,,combined dynamic monitoring of hemolytic reaction indicators can provide realtime,objective bedside auxiliary information for the efficacy assessment of LAHLH secondary to ES,and has certain clinical value in optimizing the timing of treatment adjustments.
敖正才;王慧;徐玉霞
成都市第五人民医院,四川 成都 611130成都市第五人民医院,四川 成都 611130成都市第五人民医院,四川 成都 611130
医药卫生
噬血细胞综合征埃文斯综合征溶血反应疗效评估网织红细胞
hemophagocytic lymphohistiocytosisEvans syndromehemolytic reactionefficacy assessmentreticulocyte
《中国输血杂志》 2026 (6)
789-794,6
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