Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a dysregulated immune disorder in children, associated with Epstein–Barr virus (EBV) infection or malignancies. In severe forms, HLH presents with signs and symptoms of hyperinflammation that progress to life-threatening multiorgan failure. Intervention with an extracorporeal immunomodulatory treatment utilizing a selective cytopheretic device (SCD) could be beneficial. The SCD with regional citrate anticoagulation selectively binds the most highly activated circulating neutrophils and monocytes and deactivates them before release to the systemic circulation. Multiple clinical studies, including a multicenter study in children, demonstrate SCD therapy attenuates hyperinflammation, resolves ongoing tissue injury and allows progression to functional organ recovery. We report the first case of SCD therapy in a patient with HLH and multi-organ failure.

Case diagnosis/treatment

A previously healthy 22-month-old toddler presented with fever, abdominal distension, organomegaly, pancytopenia, and signs of hyperinflammation. EBV PCR returned at > 25 million copies. The clinical and laboratory pictures were consistent with systemic EBV-positive T-cell lymphoma with symptoms secondary to HLH. The patient met inclusion criteria for an ongoing study of integration of the SCD with a continuous kidney replacement therapy (CKRT) as part of standard of care. The patient received CKRT-SCD for 4 days with normalization of serum markers of sepsis and inflammation. The patient underwent hematopoietic stem cell transplantation 52 days after presentation and has engrafted with normal kidney function 8 months later.

Conclusions

SCD treatment resulted in improvement of poor tissue perfusion reflected by rapid decline in serum lactate levels, lessened systemic capillary leak with discontinuation of vasoactive agents, and repair and recovery of lung and kidney function with extubation and removal of hemodialysis support.

中文翻译：

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体外免疫调节在患有噬血细胞性淋巴组织细胞增多症和多系统器官衰竭的幼儿中的应用

介绍

噬血细胞性淋巴组织细胞增生症 (HLH) 是一种儿童免疫失调性疾病，与 EB 病毒 (EBV) 感染或恶性肿瘤有关。在严重的情况下，HLH 会出现过度炎症的体征和症状，并发展为危及生命的多器官衰竭。利用选择性细胞分离装置 (SCD) 进行体外免疫调节治疗的干预可能是有益的。具有局部柠檬酸盐抗凝作用的 SCD 选择性地结合最高度活化的循环中性粒细胞和单核细胞，并在释放到全身循环之前使它们失活。包括儿童多中心研究在内的多项临床研究表明，SCD 疗法可减轻过度炎症、解决持续的组织损伤并促进器官功能恢复。

病例诊治

一名既往健康的 22 个月大幼儿出现发烧、腹胀、器官肿大、全血细胞减少和过度炎症迹象。EBV PCR 返回 > 2500 万份。临床和实验室图片与 HLH 继发症状的全身性 EBV 阳性 T 细胞淋巴瘤一致。该患者符合正在进行的将 SCD 与连续肾脏替代疗法 (CKRT) 整合为标准护理一部分的研究的纳入标准。患者接受 CKRT-SCD 治疗 4 天，脓毒症和炎症的血清标志物恢复正常。患者在就诊后 52 天接受了造血干细胞移植，并在 8 个月后植入正常的肾功能。

结论

SCD 治疗改善了组织灌注不良，表现为血清乳酸水平迅速下降，停用血管活性药物后全身毛细血管渗漏减少，拔管和取消血液透析支持后肺和肾功能得到修复和恢复。