Kuroki Wataru, Kobayashi Takahiro, Umakoshi Michinobu, Kitadate Akihiro, Imaizumi Chihiro, Saito Masaya, Kobayashi Isuzu, Fujishima Masumi, Fujishima Naohito, Yoshioka Tomoko, Goto Akiteru, Takahashi Naoto
Department of Hematology, Nephrology and Rheumatology, Akita University Graduate School of Medicine.
Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine.
Rinsho Ketsueki. 2022;63(6):523-529. doi: 10.11406/rinketsu.63.523.
Atraumatic splenic rupture (ASR) is a rare but fatal complication of malignant lymphoma. However, only one case of intravascular large B-cell lymphoma (IVLBCL)-related ASR (IVLBCL-ASR) has previously been reported, and the mechanism of IVLBCL-ASR is unknown. We present the case of a 78-year-old man who died unexpectedly and was diagnosed with IVLBCL-ASR pathologically by autopsy. A massive intraperitoneal hemorrhage and four lacerations on the splenic surface were discovered during the autopsy. CD20-positive lymphoma cells that infiltrated into small vessels were highly concentrated in the center of the spleen and were only slightly distributed in the lacerations on the splenic surface. Therefore, increased intrasplenic pressure due to lymphoma cell proliferation was identified as the cause of ASR. The patient had undergone F-fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT) for tongue cancer evaluation 3 months earlier, and positive uptake was found in the right adrenal gland, where lymphoma cell infiltration was confirmed by the autopsy. Our findings suggest that clinicians should be aware that the advanced stage of IVLBCL can cause fatal ASR via increased intrasplenic pressure. Therefore, early diagnosis and early treatment intervention are desirable to prevent the onset of IVLBCL-ASR, and F-FDG PET/CT is useful for the early diagnosis of IVLBCL.
非创伤性脾破裂(ASR)是恶性淋巴瘤一种罕见但致命的并发症。然而,此前仅报道过1例与血管内大B细胞淋巴瘤(IVLBCL)相关的ASR(IVLBCL-ASR),且IVLBCL-ASR的机制尚不清楚。我们报告1例78岁男性患者,其意外死亡,尸检病理诊断为IVLBCL-ASR。尸检时发现腹腔内大量出血以及脾表面有四处裂伤。浸润至小血管的CD20阳性淋巴瘤细胞高度集中于脾中央,仅少量分布于脾表面的裂伤处。因此,淋巴瘤细胞增殖导致的脾内压力升高被确定为ASR的病因。该患者3个月前曾接受F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(F-FDG PET/CT)以评估舌癌,右肾上腺发现有阳性摄取,尸检证实有淋巴瘤细胞浸润。我们的研究结果提示,临床医生应意识到IVLBCL晚期可通过脾内压力升高导致致命性ASR。因此,为预防IVLBCL-ASR的发生,早期诊断和早期治疗干预很有必要,且F-FDG PET/CT对IVLBCL的早期诊断很有用。