Noveihed Alexandra, Liang Shiochee, Glotfelty Joel, Lawrence Ibiyonu
Department of Internal Medicine, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
Discov Oncol. 2022 Mar 21;13(1):16. doi: 10.1007/s12672-022-00476-3.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease resulting from the overactivation of the immune system due to under regulation of cytotoxic lymphocytes, macrophages and natural killer (NK) cells. HLH is associated with malignancies, infections, autoimmune disorders and rarely AIDS and is rapidly fatal.
This case report identified a 53 year old male with acquired immunodeficiency syndrome (AIDS) who presented with neutropenic fever of unknown origin. He had two previous hospitalizations prior to the hospitalization diagnosing HLH. The first led to a diagnosis of drug fevers in the setting of treatment for thrombotic thrombocytopenic purpura and subsequent hospitalization led to empiric treatment of hospital acquired pneumonia after workup for intermittent fevers was negative. He was discharged but readmitted 10 days after for recurrence of neutropenic fevers. During this final hospitalization, he was found to have elevated liver enzymes, ferritin, triglycerides and soluble IL-2 receptor with persistent fevers, new splenomegaly and bicytopenia meeting the 2004 HLH criteria. Bone marrow biopsy confirmed the diagnosis of HLH as well as EBV associated large B-cell lymphoma. The patient improved on treatment with steroids, rituximab, tocilizumab, and chemotherapy but ultimately passed away due to refractory septic shock from multi-drug resistant Klebsiella pneumoniae.
This novel case highlights a patient diagnosed with HLH in the setting of several risk factors for the disease, including AIDS, B-cell lymphoma and EBV. Additionally, this case highlights the importance of early consideration of HLH in the setting of neutropenic fever without clear infectious etiology and search for malignancy associated reasons for HLH especially in immunocompromised patients.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见疾病，由于细胞毒性淋巴细胞、巨噬细胞和自然杀伤（NK）细胞调节不足导致免疫系统过度激活。HLH与恶性肿瘤、感染、自身免疫性疾病相关，与艾滋病关联罕见，且进展迅速可致命。
本病例报告了一名53岁患有获得性免疫缺陷综合征（AIDS）的男性，出现不明原因的中性粒细胞减少性发热。在诊断为HLH住院前，他曾有过两次住院经历。第一次住院因血栓性血小板减少性紫癜治疗期间诊断为药物热，随后住院在对间歇性发热检查为阴性后，经验性治疗医院获得性肺炎。他出院了，但10天后因中性粒细胞减少性发热复发再次入院。在这次最终住院期间，发现他肝酶、铁蛋白、甘油三酯和可溶性白细胞介素-2受体升高，伴有持续发热、新出现的脾肿大和双血细胞减少，符合2004年HLH标准。骨髓活检确诊为HLH以及EBV相关的大B细胞淋巴瘤。患者经类固醇、利妥昔单抗、托珠单抗和化疗治疗后病情改善，但最终因多重耐药肺炎克雷伯菌引起的难治性感染性休克死亡。
本新病例突出了一名在患有包括艾滋病、B细胞淋巴瘤和EBV等多种该疾病危险因素背景下被诊断为HLH的患者。此外，该病例突出了在中性粒细胞减少性发热且无明确感染病因的情况下早期考虑HLH的重要性，以及寻找与HLH相关的恶性肿瘤原因的重要性，尤其是在免疫功能低下的患者中。
