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,通常发生在婴儿中)或继发性(sHLH,发生在与各种疾病相关的任何年龄),主要是由感染引发的,自身免疫性疾病,和恶性状况。我们对sHLH病理生理学的理解仍在发展,以及与该综合征的原因和关联,由于这些实体的稀有性以及在报告这些实体的患者中经常出现的多种混杂因素,因此与医源性原因有关的那些被认为是最鲜为人知的。在这里,我们对文献进行了综述,以描述与医源性原因相关的sHLH的诊断和治疗挑战,并讨论了我们在更好地理解这些复杂情况以改善患者预后方面的一些挑战和未来方向.