Bains Ashish, Mamone Linda, Aneja Amandeep, Bromberg Michael
Department of Pathology and Laboratory Medicine, Temple University Hospital, 3401 N Broad Street, Philadelphia, PA 19140, USA.
Department of Hematology and Oncology, Temple University Hospital, 3401 N Broad Street, Philadelphia, PA 19140, USA.
Ann Diagn Pathol. 2017 Jun;28:37-42. doi: 10.1016/j.anndiagpath.2017.02.009. Epub 2017 Feb 20.
Secondary hemophagocytic lymphohistiocytosis (HLH) is an uncommon, but life-threatening syndrome of highly stimulated and ineffective immune dysregulation. It is not a disease entity by itself and the current diagnosis of secondary (acquired) HLH is based on constellation of nonspecific clinical and laboratory parameters indicative of overactive immune response. The presenting symptoms are often nonspecific and could potentially be missed, leading to a fatal outcome. Patients with malignancy-associated HLH have a relatively unfavorable overall survival compared with non-malignancy-associated HLH. In this retrospective study, nine adult patients with secondary HLH were identified. Of these four cases were associated with a malignancy and despite a high degree of suspicion, the underlying lymphoid malignancy was not initially evident. Three out of four patients with lymphoid malignancy-associated HLH died over a very short course of time following the diagnosis. The outcome was significantly different for the control group of patients with other underlying cause(s) for HLH. These cases emphasize the importance of a thorough search for a hidden malignant source in patients with secondary HLH for prompt diagnosis and institution of malignancy specific treatment.
继发性噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但危及生命的综合征，其特征为免疫调节高度激活但无效。它本身并非一种疾病实体，目前继发性（获得性）HLH的诊断基于一系列非特异性临床和实验室参数，这些参数表明免疫反应过度活跃。其呈现的症状往往不具特异性，可能会被漏诊，从而导致致命后果。与非恶性肿瘤相关的HLH相比，恶性肿瘤相关的HLH患者总体生存率相对较低。在这项回顾性研究中，确定了9例成年继发性HLH患者。其中4例与恶性肿瘤相关，尽管高度怀疑，但潜在的淋巴系统恶性肿瘤最初并不明显。4例淋巴系统恶性肿瘤相关HLH患者中有3例在诊断后很短时间内死亡。HLH其他潜在病因患者的对照组结果有显著差异。这些病例强调了在继发性HLH患者中彻底寻找隐匿恶性来源以进行快速诊断和开展针对恶性肿瘤的特异性治疗的重要性。
