Kumar Vaibhav, Eulitt Patrick J, Bermudez Ana, Khagi Simon
Division of Hematology & Oncology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Division of Internal Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
CNS Oncol. 2019 Dec 1;8(4):CNS45. doi: 10.2217/cns-2019-0013. Epub 2019 Nov 28.
Adult onset hemophagocytic lymphohistiocytosis (HLH) is a rare condition, usually secondary to either a precipitating infective or hematologic malignancy. We present a case of Epstein-Barr virus associated HLH in a 55-year-old female receiving treatment for a glioblastoma (GBM). It is possible that HLH is under recognized, as patients with GBM often have features of a nonspecific systemic inflammatory response syndrome, multiorgan failure and cognitive decline. A high index of suspicion and increased awareness can help improve timeliness of diagnosis. Therapeutically, Epstein-Barr virus associated HLH in patients with solid organ malignancy poses significant challenges. An individualized, multidisciplinary approach is essential when managing adult-onset HLH and providers will need to be mindful of the high mortality rate despite treatment.
成人起病的噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见疾病,通常继发于感染或血液系统恶性肿瘤。我们报告一例55岁女性胶质母细胞瘤(GBM)患者发生的与爱泼斯坦-巴尔病毒相关的HLH。HLH可能未得到充分认识,因为GBM患者常具有非特异性全身炎症反应综合征、多器官功能衰竭和认知功能下降等特征。高度的怀疑指数和提高认识有助于提高诊断的及时性。在治疗方面,实体器官恶性肿瘤患者中与爱泼斯坦-巴尔病毒相关的HLH带来了重大挑战。在管理成人起病的HLH时,个体化、多学科方法至关重要,尽管进行了治疗,但医疗人员仍需注意其高死亡率。