Zhou Huilin, Lai Shuchang, Chen Jinyi, Wang Yi, Fu Shasha, Chen Zongcun
Department of Endocrinology, The Second Affiliated Hospital of Hainan Medical University, Hainan, China.
Department of Respiratory and Critical Care Medicine, Haikou Affiliated Hospital of Central South University Xiangya School of Medicine (Haikou People's Hospital), Hainan, China.
Front Med (Lausanne). 2025 Apr 9;12:1578726. doi: 10.3389/fmed.2025.1578726. eCollection 2025.
To analyze a rare case of ANCA-associated vasculitis (AAV) complicated by hemophagocytosis and granulocytopenia induced by long-term propylthiouracil (PTU) therapy, providing insights for clinical diagnosis and management.
A retrospective analysis was conducted on the clinical data and treatment course of a patient who developed AAV with hemophagocytosis and granulocytopenia after prolonged PTU use.
Upon admission, granulocytopenia secondary to PTU was suspected. Despite transient recovery of leukocyte counts with anti-infective therapy and granulocyte colony-stimulating factor (G-CSF), recurrent leukopenia and intermittent fever persisted. Bone marrow aspiration revealed hemophagocytic cells, while serologic testing showed positivity for both PR3-ANCA and MPO-ANCA. A definitive diagnosis of PTU-induced AAV was established. Glucocorticoid therapy normalized body temperature and restored leukocyte levels. Follow-up demonstrated resolution of thyrotoxicosis, stabilized leukocyte counts, and afebrile status.
Long-term PTU therapy may trigger AAV accompanied by hemophagocytosis. Clinicians should consider screening for hemophagocytic lymphohistiocytosis (HLH) in such cases to guide timely immunosuppressive intervention.
分析1例罕见的抗中性粒细胞胞浆抗体相关性血管炎（AAV）合并噬血细胞综合征及粒细胞减少症的病例，该粒细胞减少症由长期服用丙硫氧嘧啶（PTU）引起，旨在为临床诊断和治疗提供参考。
对1例长期服用PTU后发生AAV合并噬血细胞综合征及粒细胞减少症患者的临床资料和治疗过程进行回顾性分析。
入院时，怀疑粒细胞减少症继发于PTU。尽管使用抗感染治疗和粒细胞集落刺激因子（G-CSF）后白细胞计数短暂恢复，但反复出现白细胞减少和间歇性发热。骨髓穿刺显示存在噬血细胞，而血清学检测显示PR3-ANCA和MPO-ANCA均为阳性。确诊为PTU诱导的AAV。糖皮质激素治疗使体温恢复正常，白细胞水平恢复。随访显示甲状腺毒症得到缓解，白细胞计数稳定，无发热。
长期PTU治疗可能引发AAV并伴有噬血细胞综合征。临床医生在这类病例中应考虑筛查噬血细胞性淋巴组织细胞增生症（HLH），以指导及时进行免疫抑制干预。
