一例极为罕见的结外B细胞非霍奇金淋巴瘤，累及肾上腺和心脏。
A very rare case of extranodal B-cell non-Hodgkin lymphoma presenting with adrenal and heart involvement.
作者信息
Papanastasiou Labrini, Skarakis Nikitas, Vardaka Maria, Dimitriadi Anastasia, Lampropoulou Pinelopi, Perpinia Anastasia, Vidalakis Eleftherios, Choreftaki Theodosia, Marinakis Theodoros, Kounadi Theodora, Vatalas Ioannis-Anastasios, Michalis Eurydiki
机构信息
Unit of Endocrinology and Diabetes Center, General Hospital of Athens "G. Gennimatas", Athens, Greece.
Department of Hematology, General Hospital of Athens 'G Gennimatas', Athens, Greece.
出版信息
Leuk Res Rep. 2022 Apr 27;17:100318. doi: 10.1016/j.lrr.2022.100318. eCollection 2022.
We report an extremely rare case of extranodal B-cell NHL: DLBCL (diffuse large B-cell non-Hodgkin lymphoma), stage IVE, presenting with heart and bilateral adrenal involvement. On admission, adrenal and thorax imaging identified large bilateral adrenal masses and a 4.6 cm mass in the right atrium wall. An adrenal biopsy revealed the presence of a DLBCL, with triple expression of bcl2, bcl6, C-MYC(+70%). Following six cycles of systemic immunochemotherapy with R-DA-EPOCH, and high methotrexate dose for CNS prophylaxis a marked decrease of lymphoma infiltration was observed. The selection of the appropriate treatment modality can lead to profound response and improve patient's outcome.
我们报告了一例极其罕见的结外B细胞非霍奇金淋巴瘤：弥漫性大B细胞淋巴瘤（DLBCL），IV期E组，伴有心脏和双侧肾上腺受累。入院时，肾上腺和胸部影像学检查发现双侧肾上腺有巨大肿块，右心房壁有一个4.6厘米的肿块。肾上腺活检显示存在弥漫性大B细胞淋巴瘤，bcl2、bcl6、C-MYC三重表达（阳性率70%）。在接受六个周期的R-DA-EPOCH全身免疫化疗以及高剂量甲氨蝶呤进行中枢神经系统预防后，观察到淋巴瘤浸润明显减少。选择合适的治疗方式可带来显著疗效并改善患者预后。
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