Karpova Radmila, Gorbunov Andrey, Mnatsakanyan Marina, Pogromov Aleksandr, Sokolova Irina, Shumskaya Yuliya, Russkova Ksenia, Chernousov Kirill, Momatyuk Daria
Department of Faculty Surgery No.1, University Clinical Hospital No.1, I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia.
Department of Hospital Therapy No.1, University Clinical Hospital No.1, I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia.
J Blood Med. 2021 Jun 9;12:431-434. doi: 10.2147/JBM.S283098. eCollection 2021.
Splenic marginal zone lymphoma (SMZL) is a type of non-Hodgkin's lymphoma (NL) that occurs in 2 out of 100 cases and is more common in women aged >60 years. A sluggish, asymptomatic course of the disease does not exclude transformation into a malignant form that occurs in 25% of patients with SMZL. Another equally important sign of an NL is thrombosis that occurs in 3.6% to 17.1% of the cases. In this report, we present a case of emergency splenectomy in a patient owing to difficulties in the diagnosis of SMZL, rapid onset of acute portal vein thrombosis, and the fulminant enlargement of the spleen accompanied by an increased risk of its rupture. Chronic hepatitis B was likely the trigger for transformation of the disease to an aggressive course. Portal vein thrombosis and the aggressive course of SMZL with rapid enlargement of the spleen and threat of its rupture in the background of viral hepatitis B required emergency splenectomy followed by anticoagulant, antiviral, and antitumor therapy.
脾边缘区淋巴瘤(SMZL)是一种非霍奇金淋巴瘤(NL),每100例中有2例发病,在60岁以上女性中更为常见。该病病程缓慢、无症状,但不排除有25%的SMZL患者会转变为恶性形式。NL的另一个同样重要的体征是血栓形成,发生率为3.6%至17.1%。在本报告中,我们介绍了一例患者因SMZL诊断困难、急性门静脉血栓形成迅速以及脾脏迅速肿大并伴有破裂风险增加而进行急诊脾切除术的病例。慢性乙型肝炎可能是疾病转变为侵袭性病程的触发因素。在乙型病毒性肝炎背景下,门静脉血栓形成以及SMZL的侵袭性病程伴脾脏迅速肿大和破裂风险,需要进行急诊脾切除术,随后进行抗凝、抗病毒和抗肿瘤治疗。