Kashimura Makoto, Kojima Masaru, Matsuyama Naoki, Tadokoro Jirou
Department of Hematology, Shinmatsudo Central General Hospital, Chiba, Japan.
Department of Pathology, Dokkyo Medical University, Mibu, Japan.
Pathol Res Pract. 2017 May;213(5):585-589. doi: 10.1016/j.prp.2016.12.023. Epub 2017 Jan 4.
Certain autoimmune conditions are associated with an increased risk of lymphoid malignancy. We report a 65-year old patient with autoimmune hemolytic anemia (AIHA) complicated by a follicular lymphoma (FL) in situ and other B-cell clones in the spleen. This diagnosis was made by immunohistochemistry, flow cytometry, and Southern blot analysis of the B-cell receptor. Chromosomal analysis revealed 46,XX,t(14;18)(q32;q21) 2/20, 46,XX,del(7)(q?),del(11)(q?) 2/20, and 46,XX 16/20. It has been speculated that these preneoplastic conditions do not progress to overt FL and other lymphomas without a second lymphomagenic insult. However, AIHA confers a 27.4-fold higher risk of such an insult leading to lymphoma compared with the normal healthy population. Without any therapy after splenectomy, our current study patient remained healthy with no lymphoma development for 28 months. Based on this case, we discuss the pathophysiology of lymphomagenesis in a spleen with AIHA and the roles of a splenectomy for preventing further lymphomagenesis in AIHA patients.
某些自身免疫性疾病与淋巴系统恶性肿瘤风险增加相关。我们报告了一名65岁的患者，其患有自身免疫性溶血性贫血（AIHA），并发原位滤泡性淋巴瘤（FL）以及脾脏中的其他B细胞克隆。该诊断通过免疫组织化学、流式细胞术以及B细胞受体的Southern印迹分析做出。染色体分析显示为46,XX,t(14;18)(q32;q21) 2/20、46,XX,del(7)(q?)、del(11)(q?) 2/20以及46,XX 16/20。据推测，这些肿瘤前状态若没有第二次致淋巴瘤性损伤，不会进展为明显的FL和其他淋巴瘤。然而，与正常健康人群相比，AIHA导致这种损伤进而引发淋巴瘤的风险要高27.4倍。在脾切除术后未进行任何治疗的情况下，我们目前的研究患者在28个月内保持健康，未发生淋巴瘤。基于该病例，我们讨论了AIHA患者脾脏中淋巴瘤发生的病理生理学以及脾切除术在预防AIHA患者进一步淋巴瘤发生中的作用。
