Hall David J, Dang Nam H, Shaw Christiana M
Department of Surgery, University of Florida College of Medicine, USA.
Division of Hematology & Oncology, Department of Medicine, University of Florida College of Medicine, USA.
Case Rep Surg. 2020 Feb 25;2020:6790808. doi: 10.1155/2020/6790808. eCollection 2020.
Splenic marginal zone lymphoma (SMZL) is a rare subtype of non-Hodgkin lymphoma that typically presents with symptomatic splenomegaly. The optimal treatment of SMZL not well established. A 44-year-old man with a history of low-grade B-cell lymphoproliferative disorder previously treated with chemotherapy presented with a three-month history of rapidly enlarging abdominal girth. He was found to have large symptomatic splenomegaly by computed tomography. After workup, he underwent preoperative angioembolization of the splenic artery followed by successful splenectomy. The spleen measured 45 cm in greatest dimension and weighed 12.14 kg, more than 12% of the patient's total body weight, making this both the largest spleen on record as well as the largest spleen ever surgically removed. Pathology was consistent with splenic marginal zone lymphoma. The patient did well and was discharged home on postoperative day 3.
SMZL is an infrequent condition that leads to progressive symptomatic splenomegaly which can be managed surgically providing symptomatic improvement and an overall satisfactory oncologic outcome. A multidisciplinary approach to complex cases of SMZL splenomegaly is imperative to achieving optimal outcomes.
脾边缘区淋巴瘤（SMZL）是非霍奇金淋巴瘤的一种罕见亚型，通常表现为有症状的脾肿大。SMZL的最佳治疗方法尚未明确。一名44岁男性，有低度B细胞淋巴增殖性疾病病史，曾接受化疗，出现腹部周长迅速增大3个月的症状。计算机断层扫描发现他有巨大的有症状脾肿大。经过检查，他先接受了脾动脉术前血管栓塞，随后成功进行了脾切除术。脾脏最大径为45厘米，重12.14千克，超过患者总体重的12%，这使其成为有记录以来最大的脾脏，也是有史以来手术切除的最大脾脏。病理结果与脾边缘区淋巴瘤相符。患者情况良好，术后第3天出院。
SMZL是一种罕见疾病，可导致进行性有症状脾肿大，手术治疗可改善症状并取得总体满意的肿瘤学结果。对于复杂的SMZL脾肿大病例，多学科方法对于实现最佳结果至关重要。
