Rizzi Sara, Polenzani Ilaria, Troisi Angela, Locorotondo Emanuele, Parenti Gian Carlo, Mambelli Lorenzo, Marchetti Federico
UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna - Scuola di specializzazione in Pediatria, Università di Ferrara.
UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna.
Recenti Prog Med. 2021 Oct;112(10):653-658. doi: 10.1701/3679.36656.
The paper reports the case of a 13-year-old female adolescent presenting with persistent fever. She had no other significant symptoms or signs. Laboratory examinations showed mild anemia and elevated C-reactive protein (CRP) and erythrosedimentation rate (ERS). The abdominal ultrasonography revealed para-aortic lymphadenopathy that was confirmed by magnetic resonance imaging (MRI) and positron emission tomography-computed tomography (PET/CT) that showed no other locations. The patient underwent laparoscopic excision but complete removal was not possible due to the position of the mass. The histological exam documented unicentric Castleman's disease. After surgery a clinical improvement was assisted but with persistence of very high CPR, ERS and serum amyloid. According to guidelines, she was treated with tocilizumab achieving complete remission of indices of inflammation. In the case with symptomatic unresectable unicentric Castleman's disease treatment with anti-IL-6 agents should be considered.
该论文报告了一名13岁女性青少年持续发热的病例。她没有其他明显症状或体征。实验室检查显示轻度贫血以及C反应蛋白(CRP)和红细胞沉降率(ERS)升高。腹部超声检查发现主动脉旁淋巴结肿大,磁共振成像(MRI)和正电子发射断层扫描-计算机断层扫描(PET/CT)证实了这一点,且未发现其他部位有病变。患者接受了腹腔镜切除,但由于肿块的位置无法完全切除。组织学检查记录为单中心Castleman病。手术后病情有临床改善,但CPR、ERS和血清淀粉样蛋白仍持续处于非常高的水平。根据指南,她接受了托珠单抗治疗,炎症指标完全缓解。对于有症状的不可切除单中心Castleman病病例,应考虑使用抗IL-6药物进行治疗。