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本研究旨在探讨HLH-2004方案在继发性噬血细胞性淋巴组织细胞增多症(secondary hemophagocytic lymphohistiocytosis,sHLH)患者中的临床疗效。对我科10例行HLH-2004方案化疗的sHLH患者进行回顾性分析。结果表明,化疗中7例患者有临床反应,3例患者无反应。5例sHLH患者未完成8周初始治疗,其中4例死亡,1例因严重药物副反应改用CHOP方案,治疗4个疗程后获疾病缓解。5例患者完成初始治疗,3例获疾病缓解,2例疾病控制不佳。3例缓解患者中,1例死于疾病复发,另2例维持缓解;2例控制不佳的患者中,1例死亡,另1例出院后获疾病缓解。结论:感染相关的HLH(IAHS)患者予HLH-2004方案化疗联合有效抗感染治疗后缓解率高,而EB病毒相关的HLH(EBV-HLH)或淋巴瘤相关的HLH(LAHS)患者缓解率低、缓解后易复发。
This study aimed to investigate the clinical efficacy of HLH-2004 regimen in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). We retrospectively analyzed 10 patients with sHLH who underwent HLH-2004 regimen in our department. The results showed that seven patients in the chemotherapy clinical response, three patients without response. Five patients with sHLH did not complete the initial treatment for 8 weeks, of which 4 died and one was switched to CHOP regimen due to serious side effects of drugs. After 4 courses of treatment, the disease was relieved. 5 patients completed the initial treatment, 3 patients were relieved of disease, 2 patients with poor disease control. Of the 3 patients who had remission, 1 died of disease recurrence and the other 2 patients remained remission. Of the 2 patients with poorly controlled disease, 1 died and the other 1 patient was relieved of disease after discharge. CONCLUSIONS: Infection-related HLH (IAHS) patients have a high response rate to HLH-2004 regimen combined with potent anti-infective therapy, whereas EBV-associated HLH (EBV-HLH) or lymphoma-associated HLH (LAHS) patients have a low remission rate , Ease after relapse.