Tajima Shogo, Waki Michihiko, Yamazaki Hiroyuki, Nagata Yasuyuki, Fukano Hana, Hossen Md Amir, Hoshi Shoji, Takeuchi Takahiro
Departments of Pathology, Shizuoka Saiseikai General Hospital Shizuoka, Japan.
Department of Cell Biology and Anatomy, Hamamatsu University School of Medicine Shizuoka, Japan.
Int J Clin Exp Pathol. 2014 Oct 15;7(11):8181-9. eCollection 2014.
Intravascular large B-cell lymphoma (IVLBCL), which involves the lumen of small vessels, is a rare variant of extranodal diffuse large B-cell lymphomas. Herein, we present a case of IVLBCL manifesting as cholecystitis in a 77-year-old Japanese man. He presented with fever, fatigue, and weight loss. Physical examination revealed tenderness of the right upper quadrant. The white blood cell count and C-reactive protein levels were elevated. Computed tomography revealed gallbladder thickening and pericholecystic fluid collection; these observations were consistent with the diagnosis of cholecystitis. Serum soluble interleukin-2 receptor levels were highly elevated, and gallium scintigraphy revealed an abnormal accumulation in the spleen, implying lymphoma. Consequently, G-banding analysis of the patient's bone marrow aspirates revealed the presence of different abnormal clones, including those with gain of chromosome 18 and deletion of chromosome 6q. As cholecystectomy was necessary, a concurrent splenectomy was performed to diagnose the disease definitively. Histopathologically, atypical large lymphoid cells were observed to be localized in the vasculature in both the spleen and gallbladder; the atypical cells expressed high levels of CD20, CD5, and CD10, immunohistochemically. These findings were consistent with IVLBCL. The patient underwent post-operative treatment with rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone. However, a pancreatic fistula developed during chemotherapy, causing left pleural effusion and peritoneal effusion; the patient developed sepsis from multidrug-resistant microorganisms, and subsequently died of multi-organ failure 6 months after the diagnosis. No obvious recurrence of the tumor was found during autopsy. We discuss the characteristic karyotype and immunohistochemical status observed in this case.
血管内大B细胞淋巴瘤（IVLBCL）累及小血管腔，是结外弥漫性大B细胞淋巴瘤的一种罕见变体。在此，我们报告一例77岁日本男性IVLBCL表现为胆囊炎的病例。他出现发热、乏力和体重减轻。体格检查发现右上腹压痛。白细胞计数和C反应蛋白水平升高。计算机断层扫描显示胆囊增厚和胆囊周围积液；这些表现与胆囊炎的诊断相符。血清可溶性白细胞介素-2受体水平显著升高，镓扫描显示脾脏有异常聚集，提示淋巴瘤。因此，对患者骨髓穿刺液进行G显带分析发现存在不同的异常克隆，包括18号染色体增加和6q染色体缺失的克隆。由于需要进行胆囊切除术，同时进行了脾切除术以明确诊断疾病。组织病理学检查发现，脾脏和胆囊的脉管系统中均观察到非典型大淋巴细胞；免疫组织化学检查显示，这些非典型细胞高表达CD20、CD5和CD10。这些发现与IVLBCL相符。患者术后接受了利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松龙治疗。然而，化疗期间出现胰瘘，并导致左侧胸腔积液和腹腔积液；患者因多重耐药微生物发生败血症，诊断后6个月死于多器官功能衰竭。尸检未发现肿瘤明显复发。我们讨论了该病例中观察到的特征性核型和免疫组化情况。
