Venkatesan Aadithiyavikram, Deb Rita, Nadeem Ahmed Rahim, Vidgeon Steven, Ashok Sundar, Kotsiopoulou Stella
Department of Critical Care Medicine, Croydon University Hospital, London, GBR.
Department of Haematology, Croydon University Hospital, London, GBR.
Cureus. 2025 Mar 21;17(3):e80957. doi: 10.7759/cureus.80957. eCollection 2025 Mar.
Haemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory syndrome characterised by widespread, uncontrolled T cell and histiocyte activation with accompanying cytokine storm. It can be inherited or acquired, with acquired forms triggered by infection, malignancy or autoimmune disease. There is high mortality, with patients commonly requiring critical care support. HLH is a relatively rare condition (though it is likely underdiagnosed), and there is an incomplete understanding of its pathogenesis, diagnosis and optimal management. As such, HLH presents a challenge to the critical care physician, particularly in a non-specialist centre. In our District General Hospital (DGH) intensive therapy unit, we experienced an unusually high volume of HLH cases in a short period. By presenting this case series and our improvement strategies, we aim to distil key lessons to other centres to improve the understanding of HLH for critical care physicians. There were seven confirmed cases of HLH between January 2022 and August 2024. We analysed each case, in turn, documenting the initial symptomatology, blood test results, including ferritin, and when specialist teams such as haematology and critical care became involved. We then noted when HLH treatment was started and the outcome of each case. We found that implementing a protocol to encourage early requests of an HLH blood test panel and, thereafter, early review and input by the local haematology teams, as well as critical care input for any patient with suspected HLH, improved patient outcomes: two patients died during admission pre-intervention. However, none of the patients died within their admission after our interventions. We also found that pharmacy input to ensure the emergency stock of medications used to treat HLH allowed more prompt administration of HLH treatment. Although the cornerstone of management for the critical care physician includes supportive care and organ support, HLH-specific treatments targeting the underpinning inflammation and the cytokine storm are also pivotal to controlling the disease process. Early escalation to the haematology team based on abnormally high ferritin levels and early critical care input was the cornerstone of the pathway we developed in our hospital. This system that has been developed will stand to be useful in other DGHs where HLH remains an unfamiliar condition that carries a high mortality rate.
噬血细胞性淋巴组织细胞增生症（HLH）是一种高炎症综合征，其特征是广泛的、不受控制的T细胞和组织细胞激活，并伴有细胞因子风暴。它可以是遗传性的或获得性的，获得性形式由感染、恶性肿瘤或自身免疫性疾病引发。死亡率很高，患者通常需要重症监护支持。HLH是一种相对罕见的疾病（尽管可能存在诊断不足的情况），人们对其发病机制、诊断和最佳治疗方法的了解并不完全。因此，HLH给重症监护医生带来了挑战，尤其是在非专科中心。在我们的地区综合医院（DGH）重症监护病房，我们在短时间内遇到了异常大量的HLH病例。通过展示这个病例系列和我们的改进策略，我们旨在为其他中心提炼关键经验，以提高重症监护医生对HLH的认识。在2022年1月至2024年8月期间，有7例确诊的HLH病例。我们依次分析了每个病例，记录了初始症状、血液检查结果，包括铁蛋白，以及血液学和重症监护等专科团队何时介入。然后我们记录了HLH治疗何时开始以及每个病例的结果。我们发现，实施一项协议，鼓励早期申请HLH血液检测套餐，随后由当地血液学团队进行早期评估和投入，以及对任何疑似HLH的患者进行重症监护投入，改善了患者的预后：两名患者在干预前住院期间死亡。然而，在我们进行干预后，没有患者在住院期间死亡。我们还发现，药房投入以确保用于治疗HLH的药物的应急库存，使得HLH治疗能够更迅速地进行。尽管重症监护医生的管理基石包括支持性护理和器官支持，但针对潜在炎症和细胞因子风暴的HLH特异性治疗对于控制疾病进程也至关重要。基于铁蛋白水平异常升高早期转诊至血液学团队以及早期重症监护投入是我们医院制定的治疗路径的基石。我们开发的这个系统将对其他DGH有用，在这些地方HLH仍然是一种不熟悉且死亡率高的疾病。
