原发性骨髓纤维化(PMF)是一种罕见的骨髓增殖性肿瘤,其特征是骨髓中血小板计数和纤维组织升高。JAK1/2抑制剂(JAKI),鲁索替尼,已经证明了减少脾脏大小的功效,缓解骨髓纤维化相关症状,提高总体生存率。虽然在患有PMF的患者中建议增加淋巴增生性疾病(LPD)的风险,特别是那些用JAKI治疗的人,爱泼斯坦-巴尔病毒(EBV)在此类病例中的参与仍缺乏文献记载.这里,我们介绍了一例69岁的PMF患者,该患者出现多发性淋巴结病和可溶性白细胞介素-2受体(sIL-2R)水平升高.Ruxolitinib和类固醇治疗短期改善症状;然而,淋巴结病和腹水最终恶化。活检证实EBV阳性弥漫性大B细胞淋巴瘤,但病人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EBV相关LPD进行了文献综述.我们的报告和文献综述揭示了MF中EBV相关LPD的发生,尤其是那些用JAKI治疗的人,强调需要考虑将淋巴瘤作为一种潜在的诊断方法,并在显示淋巴结病或sIL-2R水平升高的患者中监测EBV-DNA病毒载量.
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.