Hematology Institute, Meir Medical Center, Kfar Saba, Israel.
Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
J Gen Intern Med. 2022 May;37(6):1542-1546. doi: 10.1007/s11606-022-07395-7. Epub 2022 Feb 17.
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.
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综合征，可能会并发血液系统恶性肿瘤。HLH 和恶性肿瘤具有共同的临床特征，而 HLH 诊断标准（HLH-2004/Hscore）并未在该特定人群中得到验证。我们描述了一例 72 岁女性患者，她患有慢性淋巴细胞白血病已有 10 多年，病情稳定，但目前出现发热和血细胞减少。在排除了感染性病因和疾病进展后，诊断为 HLH。该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。尽管最初临床症状有所改善，但患者病情恶化，出现了肺部曲霉菌病和中枢神经系统受累，反映出 HLH 未得到控制。该患者从就诊到死亡共 45 天。该病例的一个不寻常特征是 HLH 不是由感染、疾病转化或治疗引发的。这个病例强调了区分 HLH 急剧发作与血液系统恶性肿瘤相关的其他并发症的挑战。
