没有细菌，只有细胞因子：一例癌症炎症性乳腺癌患者的免疫治疗诱导的吞噬血淋巴组织细胞增多症伪装为中性粒细胞脓毒症
No Germs, Just Cytokines: A Case of Immunotherapy-Induced Hemophagocytic Lymphohistiocytosis Masquerading as Neutropenic Sepsis in a Patient With Inflammatory Breast Cancer.
摘要(中文译文)
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的高炎症状态，可导致器官功能障碍和死亡。原发性HLH由损害免疫调节的基因突变驱动，主要影响儿童，而继发性（获得性）HLH在成人中更为常见。继发性HLH可由多种诱因引发，包括免疫检查点抑制剂（ICIs）等药物。该病的诊断较为困难，因为早期体征往往不明确且与其他疾病的表现重叠；因此，临床医生通常使用噬血细胞性淋巴组织细胞增生症-2004（HLH-2004）诊断标准和H评分来支持诊断评估。我们报告一例三阴性炎性乳腺癌患者在接受帕博利珠单抗抗癌治疗后发生HLH的病例。患者以发热和血细胞减少起病，初始按中性粒细胞减少性脓毒症进行管理。然而，在标准脓毒症治疗无反应后，对患者进行了HLH的相关检查。组织病理学和实验室检查结果支持HLH的诊断；患者符合五项HLH-2004诊断标准，并对大剂量糖皮质激素治疗有反应。本病例强调了在接受ICIs治疗的患者中，当推定诊断未如预期般产生反应时，应考虑HLH作为一种罕见的免疫相关不良事件（irAE）的重要性。它进一步说明了应用经过验证的诊断工具以加快免疫抑制治疗的价值。
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition leading to organ dysfunction and death. Primary HLH, driven by genetic mutations that impair immune regulation, predominantly affects children, whereas the secondary (acquired) form is more common in adults. Secondary HLH may be precipitated by various drivers, including medications such as immune checkpoint inhibitors (ICIs). Diagnosing this condition is difficult, as initial signs are often vague and overlap with those of other disorders; consequently, clinicians commonly use the Hemophagocytic Lymphohistiocytosis-2004 (HLH-2004) diagnostic criteria and the H-Score to support the diagnostic evaluation. We report a case of a patient with triple-negative inflammatory breast cancer who developed HLH following anticancer therapy with pembrolizumab. The patient's presentation with fever and cytopenias led to the initial management of neutropenic sepsis. However, following non-response to standard sepsis management, the patient was worked up for HLH. Diagnosis of HLH was supported by histopathological and laboratory findings; the patient fulfilled five HLH-2004 diagnostic criteria and responded to high-dose corticosteroid therapy. This case highlights the importance of considering HLH as a rare immune-related adverse event (irAE) in patients receiving ICIs when presumed diagnosis fails to respond as expected. It further illustrates the value of applying validated diagnostic tools to expedite immunosuppressive therapy.
如何引用
AMA
Nnaemeka H Ugwuchukwu. No Germs, Just Cytokines: A Case of Immunotherapy-Induced Hemophagocytic Lymphohistiocytosis Masquerading as Neutropenic Sepsis in a Patient With Inflammatory Breast Cancer.. Cureus. 2026; doi:10.7759/cureus.110906.
APA
Nnaemeka H Ugwuchukwu (2026). No Germs, Just Cytokines: A Case of Immunotherapy-Induced Hemophagocytic Lymphohistiocytosis Masquerading as Neutropenic Sepsis in a Patient With Inflammatory Breast Cancer.. Cureus. https://doi.org/10.7759/cureus.110906
GB/T 7714
Nnaemeka H Ugwuchukwu. No Germs, Just Cytokines: A Case of Immunotherapy-Induced Hemophagocytic Lymphohistiocytosis Masquerading as Neutropenic Sepsis in a Patient With Inflammatory Breast Cancer.[J]. Cureus, 2026 doi:10.7759/cureus.110906.
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