治疗与触发过度炎症：在儿科细胞治疗和重症监护环境中应对噬血细胞性淋巴组织细胞增生症及 HLH 样综合征

Hemophagocytic lymphohistiocytosis (HLH) describes a severe, hyperinflammatory syndrome that can originate from diverse etiologies, often requiring critical care level management. Primary HLH, initially described in the 1940s, derives from genetic defects that result in uncontrolled immune activation.

Although chemotherapy and immunosuppressive agents can temporarily quell inflammation, allogeneic hematopoietic cell transplantation (HCT) is the only curative option. In 2025, HCT is indicated for primary HLH and some etiologies of secondary HLH but remains challenging due to both disease and transplant-related inflammation.

Additionally, new cellular therapy approaches to treat malignancy, such as chimeric antigen receptor T cells, can trigger a spectrum of hyperinflammatory complications.

Herein, we review the pathophysiology, diagnosis, and evolving management approaches of primary and secondary HLH, ultimately informing our management of hyperinflammation in the setting of new cell therapies.