噬血细胞性淋巴组织细胞增多症 (HLH) 是一种罕见的危及生命的疾病，由组织细胞异常和 T 细胞激活引起。在成人中，它主要与感染、癌症和自身免疫性疾病有关。复发性多软骨炎 (RP) 是另一种罕见疾病，根据症状进行诊断，无需进行特殊检查，以软骨炎症为特征，表现为肿胀、发红和疼痛，很少诱发 HLH。 一名 74 岁的妇女因发烧 1 度到急诊室就诊。 38.6℃。进行血液测试、培养和成像来评估发烧情况。结果显示荧光抗核抗体水平增加和轻度血细胞减少，没有其他具体发现。影像学检查发现淋巴结肿大；然而，活检结果尚无定论。重新评估体检后，在耳朵和鼻子中观察到提示 RP 的炎症体征，需要进行组织活检进行确认。同时，持续发烧并伴有血细胞减少，促使骨髓检查发现噬血细胞。在血培养、病毒标志物和肿大淋巴结组织检查均未发现明显结果后，通过 RP 诊断为 HLH。治疗包括甲基强的松龙，然后是硫唑嘌呤。两个月后，骨髓检查证实噬血细胞增多，高铁蛋白血症和全血细胞减少正常化。彻底的体检有助于诊断和治疗不明原因发热患者中由 RP 引发的 HLH。©作者 2024。出版者百事登出版集团有限公司版权所有。

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder caused by abnormal histiocytes and T cell activation. In adults, it is predominantly associated with infections, cancers, and autoimmune diseases. Relapsing polychondritis (RP), another rare disease, is diagnosed based on symptoms without specific tests, featuring cartilage inflammation characterized by swelling, redness, and pain, rarely inducing HLH.A 74-year-old woman visited the emergency room with a fever of 38.6 °C. Blood tests, cultures, and imaging were performed to evaluate fever. Results showed increased fluorescent antinuclear antibody levels and mild cytopenia, with no other specific findings. Imaging revealed lymph node enlargement was observed; however, biopsy results were inconclusive. Upon re-evaluation of the physical exam, inflammatory signs suggestive of RP were observed in the ears and nose, prompting a tissue biopsy for confirmation. Simultaneously, persistent fever accompanied by cytopenia prompted a bone marrow examination, revealing hemophagocytic cells. After finding no significant results in blood culture, viral markers, and tissue examination of enlarged lymph nodes, HLH was diagnosed by RP. Treatment involved methylprednisolone followed by azathioprine. After two months, bone marrow examination confirmed resolution of hemophagocytosis, with normalization of hyperferritinemia and pancytopenia.Thorough physical examination enabled diagnosis and treatment of HLH triggered by RP in patients presenting with fever of unknown origin.©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.