Oncology Center, Hospital Sírio Libanês (HSL), Rua Dona Adma Jafet, 91, 2nd floor, Building A, São Paulo, SP, 01308-050, Brazil.
Division of Hematology, Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil.
J Med Case Rep. 2021 Jul 8;15(1):350. doi: 10.1186/s13256-021-02900-8.
Hemophagocytic lymphohistiocytosis is a rare, potentially fatal syndrome of immune hyperactivation. Here we describe a ganglionar tuberculosis evolving to hemophagocytic lymphohistiocytosis following adjuvant immunotherapy in a melanoma patient.
A 76-year-old Caucasian male with melanoma started with fever, diffuse petechiae, splenomegaly, anemia, thrombocytopenia, hypofibrinogenemia, and hyperferritinemia 2 months following completion of adjuvant treatment with nivolumab. Positron emission tomography scan showed significant hypermetabolism in cervical, supraclavicular, mediastinal, and abdominal lymph nodes. Bone marrow aspiration demonstrated no alterations, except for a hypercellular pattern. Dexamethasone and intravenous immunoglobulin were started owing to suspicion of hemophagocytic lymphohistiocytosis. Core biopsy of the infracarinal lymph node revealed a chronic granulomatous inflammation and caseous necrosis, with positivity for Mycobacterium tuberculosis by polymerase chain reaction, and treatment for ganglionar tuberculosis was started.
This case highlights the challenges involving programmed cell death 1 blockade in high-risk melanoma, in which infections, lymphoproliferative disorders, and sarcoidosis can mimic disease progression and trigger immune-related adverse events.
噬血细胞性淋巴组织细胞增生症是一种罕见的、潜在致命的免疫过度激活综合征。本文描述了一例黑色素瘤患者在辅助免疫治疗后发生淋巴结结核并进展为噬血细胞性淋巴组织细胞增生症。
一名 76 岁的白人男性,黑色素瘤患者,在接受纳武利尤单抗辅助治疗后 2 个月出现发热、弥漫性瘀点、脾肿大、贫血、血小板减少、低纤维蛋白原血症和高铁蛋白血症。正电子发射断层扫描显示颈、锁骨上、纵隔和腹部淋巴结有明显的高代谢。骨髓抽吸未见异常,仅表现为细胞增生。由于怀疑噬血细胞性淋巴组织细胞增生症,开始使用地塞米松和静脉注射免疫球蛋白。由于怀疑噬血细胞性淋巴组织细胞增生症,开始使用地塞米松和静脉注射免疫球蛋白。隆凸下淋巴结的核心活检显示慢性肉芽肿性炎症和干酪样坏死,聚合酶链反应检测结核分枝杆菌阳性,开始治疗结核性淋巴结炎。
本病例强调了程序性细胞死亡 1 阻断在高风险黑色素瘤中的挑战,其中感染、淋巴组织增生性疾病和结节病可模拟疾病进展并引发免疫相关不良事件。