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Natural killer/T-cell lymphoma-associated hemophagocytic syndrome: A case report

机译:自然杀手/ T细胞淋巴瘤相关的噬血细胞综合征:一例报告

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摘要

Natural killer (NK)/T-cell lymphoma-associated hemophagocytic syndrome (HPS) is a rare and fatal disease with no optimal treatment. The present study reports the clinical features, diagnosis and treatment process of three patients with relapsed NK/T-cell lymphoma-associated HPS. All of the patients were classified as Ann Arbor stage IV and presented with a poor performance status. Two patients were successfully treated with a pegaspargase-containing combination regimen and one patient succumbed due to serious complications. These cases indicate that for patients with a history of lymphoma, the diagnosis of HPS should be considered when patients present with progressive high fever, pancytopenia and liver dysfunction. Early identification and effective treatments, including pegaspargase-based regimens are essential for an enhanced prognosis.
机译:自然杀手(NK)/ T细胞淋巴瘤相关的噬血细胞综合征(HPS)是一种罕见且致命的疾病,没有最佳治疗方法。本研究报告了三例复发的NK / T细胞淋巴瘤相关HPS的临床特征,诊断和治疗过程。所有患者均被归为Ann Arbor IV期,表现为较差的表现。 2例患者接受了含PEGA精酶的联合疗法成功治疗,1例患者因严重并发症而屈服。这些病例表明,对于有淋巴瘤病史的患者,当出现进行性高烧,全血细胞减少症和肝功能异常的患者时,应考虑HPS的诊断。早期识别和有效治疗,包括基于pegaspargase的治疗方案,对于提高预后至关重要。

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