Briand P Y, Gangneux J P, Favaretto G, Ly-Sunnaram B, Godard M, Robert-Gangneux F, Fest T
Laboratoire d'hématologie-immunologie-thérapie cellulaire, CHU de Pontchaillou, Rennes.
Ann Biol Clin (Paris). 2008 Mar-Apr;66(2):199-205. doi: 10.1684/abc.2008.0209.
Hemophagocytic syndrome (HPS) is a clinical entity that combines the clinical, biological and histological symptoms. The physiopathological mechanism involves the interaction between T lymphocytes/NK cells and macrophages, at the origin of an uncontrolled activation of the macrophages. The consequence is a hemophagocytosis extending to numerous organs, preferentially bone marrow. Clinical symptoms include cytopenia, fever unresponsive to antibiotics and multiple organ dysfunctions. Infections, lymphoproliferative disorders, cancers, systemic diseases are the most prevalent triggers or etiologies of HPS. Because of its high risk of mortality, HPS constitutes a diagnostic and therapeutic urgency. The search for an aetiology, in particular by serological testing, is essential because it conditions the treatment and thus the evolution of the disease. We report here the case of a 12 years-old boy presenting a HPS secondary to a toxoplasmic primo-infection. The objective of this work is to present the step of the biological diagnosis of HPS. Moreover, this observation allows the study of a very rare clinical presentation of toxoplasmic primo-infection, in an immunocompetant patient.
噬血细胞综合征(HPS)是一种综合了临床、生物学和组织学症状的临床实体。其生理病理机制涉及T淋巴细胞/NK细胞与巨噬细胞之间的相互作用,导致巨噬细胞不受控制地激活。结果是噬血细胞现象扩展到多个器官,尤其是骨髓。临床症状包括血细胞减少、对抗生素无反应的发热和多器官功能障碍。感染、淋巴增殖性疾病、癌症、全身性疾病是HPS最常见的触发因素或病因。由于其高死亡率风险,HPS构成了诊断和治疗的紧迫性。寻找病因,特别是通过血清学检测,至关重要,因为它决定了治疗方案,进而影响疾病的发展。我们在此报告一例12岁男孩因初次弓形虫感染继发HPS的病例。这项工作的目的是介绍HPS生物学诊断的步骤。此外,该病例有助于研究免疫功能正常患者中非常罕见的初次弓形虫感染临床表现。