噬血细胞性淋巴组织细胞增多症 (HLH) 是一种高炎症性疾病，可以是家族性的，也可以是获得性的，如果不治疗，通常会导致多器官衰竭和死亡。 HLH 的治疗通常需要结合糖皮质激素和细胞毒性化疗。我们描述了一位女性的病例，她出现了 HLH 的体征和症状，后来发现她患有原发性中枢神经系统 (CNS) 弥漫性大 B 细胞淋巴瘤。她的 HLH 症状通过高剂量地塞米松成功治疗，她的原发性中枢神经系统淋巴瘤则通过高剂量甲氨蝶呤和利妥昔单抗治疗。这是继发于原发性 CNS 淋巴瘤的罕见 HLH 病例，其中 HLH 仅用类固醇进行控制，不需要使用基于依托泊苷的治疗方案或环磷酰胺、多柔比星、长春新碱和强的松。© BMJ Publishing Group Limited 2024。无商业报道-使用。请参阅权利和权限。英国医学杂志出版。

Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition that can be either familial or acquired and, if untreated, frequently results in multiorgan failure and death. Treatment of HLH typically requires a combination of glucocorticoids and cytotoxic chemotherapy. We describe the case of a woman who presented with signs and symptoms concerning for HLH who was later found to have a primary central nervous system (CNS) diffuse large B-cell lymphoma. Her HLH symptoms were successfully treated with high doses of dexamethasone, and her primary CNS lymphoma was treated with high-dose methotrexate and rituximab. This is a rare case of HLH secondary to primary CNS lymphoma where HLH was controlled with steroids alone and did not require the use of an etoposide-based regimen or cyclophosphamide, doxorubicin, vincristine and prednisone.© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.