期刊
ACTA HAEMATOLOGICA
卷 -, 期 -, 页码 -出版社
KARGER
DOI: 10.1159/000535449
关键词
allogeneic hematopoietic cell transplantation (HCT); hemophagocytic lymphohistiocytosis (HLH); relapse; donor lymphocyte infusion (DLI)
类别
We present a case of a 29-year-old male with primary HLH that relapsed after HCT and achieved durable long disease-free survival following a donor-lymphocyte infusion (DLI), demonstrating the efficacy of DLI for relapsed primary HLH.
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.
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