Abstract：
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.
摘要：
噬血细胞性淋巴组织细胞增多症（HLH）是一种可以是家族性或获得性的高炎症，如果不治疗,经常导致多器官衰竭和死亡。HLH的治疗通常需要糖皮质激素和细胞毒性化疗的组合。我们描述了一名妇女的病例，该妇女出现有关HLH的体征和症状，后来被发现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症状被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接受大剂量甲氨蝶呤和利妥昔单抗治疗。这是原发性中枢神经系统淋巴瘤继发HLH的罕见病例，其中HLH仅由类固醇控制，不需要使用基于依托泊苷的方案或环磷酰胺。阿霉素,长春新碱和泼尼松.
