Olin Rebecca L, Nichols Kim E, Naghashpour Mojdeh, Wasik Mariusz, Shelly Brenda, Stadtmauer Edward A, Vogl Dan T
Hematologic Malignancies Program, Abramson Cancer Center, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA.
Am J Hematol. 2008 Sep;83(9):747-9. doi: 10.1002/ajh.21236.
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe inflammatory disorder marked by abnormal cytotoxic T and natural killer cell activity, resulting in impaired clearance of pathogen, excessive cytokine production, and continued immune system activation. Soluble IL-2 receptor (sIL-2R or sCD25) is typically elevated in HLH and can serve as a marker of disease activity, although its role in the pathophysiology of the disease is unclear. Here we present a case of an adult patient with steroid-dependent HLH who was treated successfully with daclizumab, a monoclonal anti-CD25 antibody, allowing successful withdrawal of steroid therapy without an increase in symptoms.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见且严重的炎症性疾病，其特征为细胞毒性T细胞和自然杀伤细胞活性异常，导致病原体清除受损、细胞因子过度产生以及免疫系统持续激活。可溶性白细胞介素-2受体（sIL-2R或sCD25）在HLH中通常会升高，可作为疾病活动的标志物，尽管其在该疾病病理生理学中的作用尚不清楚。在此，我们报告一例成年类固醇依赖型HLH患者，该患者接受抗CD25单克隆抗体达利珠单抗治疗成功，得以成功停用类固醇疗法且未出现症状加重。
