Noversa de Sousa Rita, Sá Lima Andreia, Viana Susana, Guimarães Filipa, Pereira Marta, Afonso Luís Miguel
Internal Medicine Service, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, Portugal.
Intensive Care Unit, Pedro Hispano Hospital, Matosinhos Local Health Unit, Matosinhos, Portugal.
Eur J Case Rep Intern Med. 2024 Dec 12;11(12):005040. doi: 10.12890/2024_005040. eCollection 2024.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome marked by excessive immune activation. It can be triggered by various factors, including infections, malignancies, and autoimmune diseases, making the diagnosis challenging due to its overlap with other severe conditions.
We discuss two intensive care unit (ICU) cases illustrating the diverse manifestations of HLH and the critical importance of early recognition and treatment. The first case involves natural killer-cell leukaemia, and the second, a suspected viral trigger. Both highlight the necessity of a multidisciplinary approach in diagnosis and management, emphasizing the complexity of HLH in ICU settings.
High mortality rates, particularly in malignancy-associated HLH, underscore the importance of tailored treatment strategies based on the underlying aetiology.
Hemophagocytic lymphohistiocytosis (HLH) in adults can arise from a variety of triggers, including infections and malignancies, each influencing disease progression and prognosis differently. Recognizing these underlying aetiologies is crucial for tailoring management strategies and anticipating clinical outcomes.Due to its life-threatening nature, HLH requires prompt diagnosis and a coordinated, multidisciplinary approach. Early intervention, incorporating immunosuppressive therapies and supportive care, is essential to improve patient outcomes, particularly in intensive care unit settings where disease severity is often pronounced.Utilizing diagnostic tools such as the HScore and HLH-2004 criteria can facilitate early identification of HLH in critically ill patients with unexplained inflammatory symptoms. These tools, along with a high index of suspicion, help distinguish HLH from other hyperinflammatory conditions, enabling timely and appropriate therapeutic interventions.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的、危及生命的高炎症综合征，其特征为过度的免疫激活。它可由多种因素触发，包括感染、恶性肿瘤和自身免疫性疾病，因其与其他严重病症存在重叠，故诊断具有挑战性。
我们讨论两例重症监护病房（ICU）病例，以说明HLH的多样表现以及早期识别和治疗的至关重要性。第一例涉及自然杀伤细胞白血病，第二例怀疑由病毒触发。两者均凸显了多学科方法在诊断和管理中的必要性，强调了ICU环境中HLH的复杂性。
高死亡率，尤其是在与恶性肿瘤相关的HLH中，凸显了基于潜在病因制定个性化治疗策略的重要性。
成人噬血细胞性淋巴组织细胞增生症（HLH）可由多种触发因素引起，包括感染和恶性肿瘤，每种因素对疾病进展和预后的影响各不相同。识别这些潜在病因对于制定管理策略和预测临床结果至关重要。由于其危及生命的性质，HLH需要迅速诊断和协调的多学科方法。早期干预，包括免疫抑制治疗和支持性护理，对于改善患者预后至关重要，尤其是在疾病严重程度通常较高的重症监护病房环境中。使用诸如HScore和HLH - 2004标准等诊断工具，有助于在患有不明原因炎症症状的重症患者中早期识别HLH。这些工具以及高度的怀疑指数，有助于将HLH与其他高炎症病症区分开来，从而实现及时和适当的治疗干预。
