Department of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Department of Cardiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
BMC Cardiovasc Disord. 2022 Feb 4;22(1):31. doi: 10.1186/s12872-022-02480-5.
Burkitt lymphoma (BL) is a rare disease with the sporadic variant accounting for less than 1% of adult non-Hodgkin lymphomas. BL usually presents with an abdominal bulk, but extranodal disease affecting the bone marrow and central nervous system is common. Cardiac manifestations, however, are exceedingly rare, with less than 30 cases reported in the literature.
We report on a 54-year-old male patient with a six week-long history of paranasal sinus swelling, fatigue and dyspnea on exertion. Stage IV sporadic BL with extensive lymphonodal and cardiovascular involvement was diagnosed. Manifestations included supra- and infradiaphragmatic lymphadenopathy as well as infiltration of the aortic root, the pericardium, the right atrium and the right ventricle. EBV-reactivation was detected, which is uncommon in the sporadic subtype. After initial full-dose chemotherapy with very good BL control, the patient developed acute, but fully reversible cardiac insufficiency. Myocardial lymphoma involvement receded completely during the following two therapy cycles, while cardiac function periodically deteriorated shortly after chemotherapy administration and quickly recovered thereafter. Interestingly, the decline in cardiac function lessened with decreasing myocardial lymphoma manifestation. Once the cardiovascular BL infiltration was resolved, cardiac function remained stable throughout further treatment. Following seven cycles of chemotherapy and mediastinal radiation, the patient is now in continued complete remission.
Although rare, cardiac involvement in BL can quickly become life-threatening due to rapid lymphoma doubling time and should therefore be considered at initial diagnosis. This case suggests an association between myocardial infiltration, chemotherapy associated tumor cell lysis and transient deterioration of cardiac function until the damage caused by the underlying lymphoma could be restored. While additional studies are needed to further elucidate the mechanisms of acute cardiac insufficiency due to lymphoma lysis in the infiltrated structures, prompt BL control and full recovery of the patient supports courageous treatment start despite extensive cardiovascular involvement.
伯基特淋巴瘤（BL）是一种罕见疾病，散发性变异占成人非霍奇金淋巴瘤的比例不到 1%。BL 通常表现为腹部肿块，但结外疾病累及骨髓和中枢神经系统很常见。然而，心脏表现极为罕见，文献中报告的病例少于 30 例。
我们报告了一名 54 岁男性患者，病史为 6 周的鼻窦肿胀、疲劳和运动时呼吸困难。诊断为 IV 期散发性 BL，广泛累及淋巴结和心血管系统。表现包括膈上和膈下淋巴结病以及主动脉根部、心包、右心房和右心室浸润。检测到 EBV 再激活，这在散发性亚型中不常见。在初始全剂量化疗后 BL 得到很好的控制，但患者随后出现急性但完全可逆的心力衰竭。心肌淋巴瘤受累在随后的两个治疗周期中完全消退，而在化疗给药后不久，心脏功能周期性恶化，此后迅速恢复。有趣的是，心脏功能的下降随着心肌淋巴瘤表现的减少而减轻。一旦心血管 BL 浸润得到解决，心脏功能在整个后续治疗过程中保持稳定。经过 7 个周期的化疗和纵隔放疗，患者目前处于持续完全缓解状态。
尽管罕见，但由于淋巴瘤倍增时间快，BL 心脏受累可能很快危及生命，因此应在初始诊断时考虑。本病例提示心肌浸润、化疗相关肿瘤细胞溶解与心脏功能短暂恶化之间存在关联，直至潜在淋巴瘤造成的损伤得到恢复。虽然需要进一步研究来进一步阐明浸润结构中淋巴瘤溶解导致急性心力衰竭的机制，但 BL 迅速得到控制和患者完全恢复支持在广泛心血管受累的情况下勇敢地开始治疗。
