Oshima Seigi, Inano Shojiro, Kitano Toshiyuki
Hematology, Kitano Hospital Medical Research Institute, Osaka, JPN.
Hematology and Oncology, Kyoto University Hospital, Kyoto, JPN.
Cureus. 2024 Mar 20;16(3):e56586. doi: 10.7759/cureus.56586. eCollection 2024 Mar.
Primary myelofibrosis (PMF) is a rare myeloproliferative neoplasm characterized by elevated platelet counts and fibrous tissues in the bone marrow. The JAK1/2 inhibitor (JAKi), ruxolitinib, has demonstrated efficacy in reducing splenic size, alleviating myelofibrosis-related symptoms, and improving overall survival. While an increased risk of lymphoproliferative disease (LPD) is suggested in patients with PMF, particularly those treated with JAKi, the involvement of Epstein-Barr virus (EBV) in such cases remains poorly documented. Here, we present the case of a 69-year-old woman with PMF who developed multiple lymphadenopathies and elevated soluble interleukin-2 receptor (sIL-2R) levels. Ruxolitinib and steroid therapy improved the symptoms for a short period; however, the lymphadenopathies and ascites eventually worsened. A biopsy confirmed EBV-positive diffuse large B-cell lymphoma, but the patient died of severe tumor lysis syndrome. Additionally, we conducted a literature review on EBV-related LPD in patients with primary and secondary myelofibrosis. Our report and literature review shed light on the occurrence of EBV-related LPD in MF, especially in those treated with JAKi, emphasizing the need to consider lymphoma as a potential diagnosis and monitor the EBV-DNA viral load in patients displaying lymphadenopathies or increased sIL-2R levels.
原发性骨髓纤维化（PMF）是一种罕见的骨髓增殖性肿瘤，其特征为血小板计数升高以及骨髓中出现纤维组织。JAK1/2抑制剂（JAKi）鲁索替尼已被证明在缩小脾脏大小、缓解骨髓纤维化相关症状以及改善总生存期方面具有疗效。虽然提示PMF患者，尤其是接受JAKi治疗的患者发生淋巴增殖性疾病（LPD）的风险增加，但爱泼斯坦-巴尔病毒（EBV）在这类病例中的参与情况仍鲜有文献记载。在此，我们报告一例69岁患有PMF的女性患者，该患者出现多处淋巴结病且可溶性白细胞介素-2受体（sIL-2R）水平升高。鲁索替尼和类固醇治疗在短期内改善了症状；然而，淋巴结病和腹水最终恶化。活检证实为EBV阳性弥漫性大B细胞淋巴瘤，但患者死于严重的肿瘤溶解综合征。此外，我们对原发性和继发性骨髓纤维化患者中与EBV相关的LPD进行了文献综述。我们的报告和文献综述揭示了MF中与EBV相关的LPD的发生情况，尤其是在接受JAKi治疗的患者中，强调需要将淋巴瘤视为一种潜在诊断，并监测出现淋巴结病或sIL-2R水平升高的患者的EBV-DNA病毒载量。
