Department of Pathology, Cancer Research Center, Faculty of Medicine, Tishreen University, Lattakia, Syria.
Faculty of Medicine, Tishreen University, Lattakia, Syria.
J Med Case Rep. 2021 May 6;15(1):227. doi: 10.1186/s13256-021-02846-x.
Primary splenic lymphoma represents a rare entity that constitutes less than 1% of non-Hodgkin lymphomas, and less than 2% of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of primary splenic lymphomas. DLBCL encompasses a heterogeneous entity with distinct morphological variants. The anaplastic variant of DLBCL was first defined in the 2017 World Health Organization classification as a rare histological subtype that constitutes less than 3.4% of DLBCL cases.
A 65-year-old Syrian man presented to our hospital with constant dull localized left upper quadrant abdominal pain for about 20 days accompanied by general weakness, loss of appetite, and rapid weight loss. Clinical examination revealed isolated splenomegaly and left upper abdominal tenderness. Following physical, laboratory, and radiologic examinations, the patient underwent splenectomy. Interestingly, pathological and immunohistochemical examinations of the resected spleen confirmed the diagnosis of a primary anaplastic variant of DLBCL.
Herein, we aimed to present an unusual combination of a rare splenic neoplasm and a unique lymphoma subtype. Furthermore, we aimed to highlight the difficulties in differential diagnosis and the importance of histological and immunohistochemical examinations with clinical correlation.
原发性脾脏淋巴瘤是一种罕见的疾病，占非霍奇金淋巴瘤的比例不到 1%，占所有淋巴瘤的比例不到 2%。弥漫性大 B 细胞淋巴瘤（DLBCL）是原发性脾脏淋巴瘤最常见的组织学亚型。DLBCL 包含一个具有不同形态学变异的异质性实体。间变性 DLBCL 于 2017 年世界卫生组织分类中首次被定义为一种罕见的组织学亚型，占 DLBCL 病例的比例不到 3.4%。
一名 65 岁的叙利亚男性因左上腹持续钝痛约 20 天就诊于我院，伴有全身乏力、食欲不振和体重迅速下降。体格检查发现孤立性脾肿大和左上腹压痛。经过体格、实验室和影像学检查，患者接受了脾切除术。有趣的是，切除脾脏的病理和免疫组织化学检查证实了原发性间变性 DLBCL 的诊断。
本研究旨在介绍一种罕见脾脏肿瘤与独特淋巴瘤亚型的罕见组合。此外，本研究旨在强调鉴别诊断的困难以及与临床相关性的组织学和免疫组织化学检查的重要性。
