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医源性噬血细胞性淋巴组织细胞增生症。

Iatrogenic HLH.

机构信息

Department of Haematology, Imperial College London, London, UK.

Department of Immunology, Cleveland Clinic, Cleveland, OH, USA.

出版信息

Adv Exp Med Biol. 2024;1448:469-477. doi: 10.1007/978-3-031-59815-9_32.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) can be categorized as either primary (familial, generally occurring in infants) or secondary (sHLH, occurring at any age in association with a variety of conditions) and is mainly triggered by infections, autoimmune diseases, and malignant conditions. Our understanding of the pathophysiology of sHLH is still evolving, and among the causes and associations with the syndrome, those putatively associated with iatrogenic causes remain among the most poorly understood due to the rarity of these entities and the multiple confounders so often present in the patients in whom they are reported. Herein, we present a review of the literature to describe the diagnostic and therapeutic challenges of sHLH associated with iatrogenic causes and discuss some of the challenges and future directions in our efforts to better understand these complex conditions for the advancement of patient outcomes.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)可分为原发性(家族性,通常发生在婴儿期)或继发性(sHLH,可在任何年龄发生,并与多种疾病相关),主要由感染、自身免疫性疾病和恶性疾病引发。我们对 sHLH 的病理生理学的理解仍在不断发展,在该综合征的病因和关联中,那些推测与医源性原因相关的仍然是最不为人理解的,因为这些实体很少见,而且在报告这些实体的患者中常常存在多种混杂因素。在此,我们对文献进行了回顾,以描述与医源性原因相关的 sHLH 的诊断和治疗挑战,并讨论了在努力更好地理解这些复杂疾病以改善患者预后方面所面临的一些挑战和未来方向。

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