Renal Division, Department of Medicine, Peking University First Hospital; Peking University Institute of Nephrology, Key Laboratory of Renal Disease, Ministry of Health of China; and Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, 8 Xishiku Street; Xicheng District, Beijing, China.
BMC Nephrol. 2019 Jan 29;20(1):27. doi: 10.1186/s12882-019-1217-1.
Hemophagocytic lymphohistiocytosis (HLH) is characterized by excessive activation of the immune system due to infection, autoimmune diseases, or malignancy. As an aggressive and life-threatening clinical syndrome, HLH secondary to peritoneal dialysis associated peritonitis (PDAP) has never been reported.
A 34-year-old female peritoneal dialysis (PD) patient was hospitalized for fever, progressively multi-organ damage (including cytopenias, abnormalities of coagulation and liver enzyme) after an episode of organism-specific peritonitis. She was refractory to the broad-spectrum antimicrobial agent. Further tests found hemophagocytosis on the bone marrow examination, and extremely high level of sIL2-R and impaired activity of NK cell. The diagnosis of HLH was eventually established. After HLH-specific therapy, this patient recovered and discharged.
The present case suggests that clinicians should to be aware of HLH in those patients apparently suspected with refractory or relapsing peritonitis, especially those accompanied with persist fever, hyperferritinemia, and cytopenias. HLH-specific therapy and supportive care should be applied without delay.
噬血细胞性淋巴组织细胞增生症（HLH）的特征是由于感染、自身免疫性疾病或恶性肿瘤导致免疫系统过度激活。HLH 作为一种侵袭性和危及生命的临床综合征，继发于腹膜透析相关性腹膜炎（PDAP）的情况尚未见报道。
一名 34 岁女性 PD 患者因腹膜炎发作后出现发热和进行性多器官损伤（包括血细胞减少、凝血和肝酶异常）而住院。她对抗生素治疗反应不佳。进一步的检查发现骨髓检查中有噬血现象，可溶性白细胞介素 2 受体（sIL2-R）水平极高，NK 细胞活性受损。最终诊断为 HLH。经 HLH 特异性治疗后，该患者康复出院。
本病例提示临床医生应警惕那些明显疑似难治性或复发性腹膜炎的患者出现 HLH，尤其是那些伴有持续发热、高血清铁蛋白血症和血细胞减少的患者。应立即应用 HLH 特异性治疗和支持性护理。
