Primary hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory disorder characterized by dysregulation of inflammatory cells and cytokine signalling. Although first-line treatment consisting of immunosuppressive therapy and allogeneic hematopoietic cell transplantation (HCT) is often curative, it remains unknown whether any effective therapies exist for disease relapse/progression after HCT. Here we present a case of a 29-year-old male with primary HLH that relapsed after HCT and subsequently achieved durable long disease-free survival following a donor-lymphocyte infusion (DLI). To our knowledge, this represents the first case demonstrating the efficacy of DLI for relapsed primary HLH.

中文翻译：

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对同种异体造血细胞移植后原发性噬血细胞性淋巴组织细胞增多症复发的供体淋巴细胞输注完全缓解。

原发性噬血细胞性淋巴组织细胞增多症 (HLH) 是一种高炎症性疾病，其特征是炎症细胞和细胞因子信号传导失调。尽管由免疫抑制治疗和同种异体造血细胞移植（HCT）组成的一线治疗通常可以治愈，但对于 HCT 后疾病复发/进展是否存在有效的治疗方法仍不清楚。在这里，我们介绍了一名患有原发性 HLH 的 29 岁男性病例，该患者在 HCT 后复发，随后在供体淋巴细胞输注 (DLI) 后实现了持久的长期无病生存。据我们所知，这是第一个证明 DLI 对复发性原发性 HLH 有效的病例。