Magomedova A U, Fastova E A, Kovrigina A M, Obukhova T N, Skidan N I, Mangasarova Ya K, Vorobyev A I, Kravchenko S K
National Research Center for Hematology, Ministry of Health of Russia, Moscow, Russia.
Ter Arkh. 2017;89(7):65-68. doi: 10.17116/terarkh201789765-68.
Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct type of large B-cell lymphoma. In this type of the disease, the neoplastic process is located in the anterior and superior mediastinum, frequently with compression of the superior vena cava and with tumor invasion into the adjacent organs and tissues: the pericardium, lung, pleura, etc. Despite the fact that in PMBCL progression, there may be involvement of extranodal organs, such as the kidney, adrenal glands, liver, and central nervous system, bone marrow (BM) injury is generally absent. Since BM injury in patients with diffuse large B-cell lymphoma is an independent poor prognostic indicator, there is reason to believe that BM involvement in PMBCL affects the prognosis. These cases may need intensified induction therapy followed by autologous hematopoietic stem cell transplantation; and BM injury should be monitored during the therapy. The paper gives reports of clinical cases of bone marrow involvement in 2 PMBCL patients treated at the National Research Center for Hematology, Ministry of Health of the Russian Federation.
原发性纵隔大B细胞淋巴瘤(PMBCL)是一种独特类型的大B细胞淋巴瘤。在这种疾病类型中,肿瘤形成过程位于前上纵隔,常伴有上腔静脉受压以及肿瘤侵犯相邻器官和组织:心包、肺、胸膜等。尽管在PMBCL进展过程中可能会累及结外器官,如肾脏、肾上腺、肝脏和中枢神经系统,但通常不存在骨髓(BM)损伤。由于弥漫性大B细胞淋巴瘤患者的BM损伤是一个独立的不良预后指标,因此有理由认为PMBCL中的BM累及会影响预后。这些病例可能需要强化诱导治疗,随后进行自体造血干细胞移植;并且在治疗期间应监测BM损伤情况。本文报道了俄罗斯联邦卫生部国家血液学研究中心治疗的2例PMBCL患者骨髓受累的临床病例。