A Case of Chronic Lymphocytic Leukemia Complicated by Hemophagocytic Lymphohistiocytosis: Identifying the Aberrant Immune Response.
A Case of Chronic Lymphocytic Leukemia Complicated by Hemophagocytic Lymphohistiocytosis: Identifying the Aberrant Immune Response.
复制标题
慢性淋巴细胞白血病并发噬血细胞性淋巴组织细胞增多症一例：识别异常的免疫反应。
DOI：
10.1007/s11606-022-07395-7
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发表时间：
2022
影响因子：
5.7
通讯作者：
Ellis,Martin
中科院分区：
文献类型：
--
作者：
Zoref-Lorenz,Adi;Yuklea,Mona;Topaz,Guy;Jordan,MichaelB;Ellis,Martin
噬血细胞淋巴组织细胞增多症是一种危及生命的高炎症综合征，可能会使血液系统的恶性肿瘤复杂化。人类促黄体生成素和恶性肿瘤有共同的临床特征，而人类促黄体生成素诊断标准(HLH-2004/Hcore)在这一特定人群中并未得到验证。我们描述一位72岁的慢性淋巴细胞性白血病病史稳定超过10年的病人，他表现为发热和细胞减少。在排除感染病因和疾病进展后，诊断为HLH。患者接受了依托泊苷、地塞米松、静脉注射免疫球蛋白和利妥昔单抗治疗。尽管最初的临床症状有所改善，但患者病情恶化并发展为肺曲霉菌病和中枢神经系统受累，这反映了HLH的失控。患者在发病45天后死亡。该病例的一个不同寻常的特征是，HLH不是由感染、疾病转化或治疗引发的。本病例强调了与血液系统恶性肿瘤相关的其他并发症与压倒性HLH的发展相区别的挑战。
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that may complicate hematologic malignancies. HLH and malignancies have common clinical features, and HLH diagnostic criteria (HLH-2004/Hscore) were not validated in this specific population. We describe a case of a 72-year-old patient with a history of chronic lymphocytic leukemia stable for over 10 years who presented with fever and cytopenia. After excluding infectious etiologies and the progression of her disease, HLH was diagnosed. The patient was treated with etoposide, dexamethasone, intravenous immunoglobulin, and rituximab. Despite initial clinical improvement, the patient deteriorated and developed pulmonary aspergillosis and CNS involvement that reflected uncontrolled HLH. The patient died 45 days after her presentation. An unusual feature of this case was that HLH was not triggered by infection, disease transformation, or treatment. This case emphasizes the challenges of differentiating the development of overwhelming HLH from other complications associated with hematologic malignancy.