Makusha Lovemore Peter, Pucar Darko, Young Colin R
Department of Radiology and Imaging, Yale University School of Medicine, New Haven, CT, USA.
World J Nucl Med. 2020 Jul 2;19(3):281-283. doi: 10.4103/wjnm.WJNM_73_19. eCollection 2020 Jul-Sep.
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare disease with either an indolent or aggressive course. A 29-year-old male presented with fever, polyarthralgias, splenomegaly, retroperitoneal adenopathy, and laboratory findings consistent with Epstein-Barr-mediated sHLH. Consistent with a prior survival analysis by Kim ., splenic maximum standardized uptake value (SUV) >2.52 and bone marrow SUV >3.13 on F fuorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) predicted an aggressive disease with poor treatment response. Despite optimal treatment, the patient rapidly progressed to death within 3 months of symptom onset. This case underscores the potential lethal nature of sHLH, and the evolving role of FDG-PET/CT in predicting disease severity and treatment response.
继发性噬血细胞性淋巴组织细胞增生症（sHLH）是一种病程隐匿或侵袭性的罕见疾病。一名29岁男性出现发热、多关节痛、脾肿大、腹膜后淋巴结肿大，实验室检查结果符合爱泼斯坦-巴尔病毒介导的sHLH。与Kim等人之前的生存分析一致，氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描（FDG-PET/CT）显示脾脏最大标准化摄取值（SUV）>2.52以及骨髓SUV>3.13预示疾病侵袭性强且治疗反应差。尽管进行了最佳治疗，患者在症状出现后3个月内迅速进展至死亡。该病例强调了sHLH潜在的致死性，以及FDG-PET/CT在预测疾病严重程度和治疗反应方面不断演变的作用。
