Ogawa Maiko Angela, Ieda Miho, Hayakawa Masaya, Mizutani Hiroyuki, Ogawa Kenichiro, Sato Tomoko, Satake Tatsunari, Kojima Yumi
Department of Hematology, Nagoya Ekisaikai Hospital.
Department of Emergency Medicine, Nagoya Ekisaikai Hospital.
Rinsho Ketsueki. 2022;63(3):182-188. doi: 10.11406/rinketsu.63.182.
Intravascular large B-cell lymphoma (IVLBCL) is a rare form of non-Hodgkin B-cell lymphoma which occurs mainly in capillaries and small blood vessels. Successful diagnosis of IVLBCL is challenging since it lacks tumor formation and presents various clinical manifestations. An 82-year-old Asian female patient presented to our emergency department with a history of general fatigue, weight loss, and fever for two weeks. The patient's random skin biopsy was negative, and her bone marrow biopsy revealed hemophagocytic syndrome with no obvious involvement of lymphoma cells. Gallium scintigraphy showed mild uptake in the uterus, pelvis, and spine. The repetitive bone marrow biopsy result and the endometrial cytology/biopsy were negative; however, the pelvic MRI was compatible with lymphoma, revealing lesions in the corpus uteri, pelvis, and vertebral body. After laparoscopic-assisted vaginal total hysterectomy and bilateral salpingo-oophorectomy, the diagnosis of the Asian variant of IVLBCL was made. Although total hysterectomy remains controversial for elderly patients with declining performance status, we could successfully diagnose the condition and initiate the treatment. The patient's general condition improved soon after starting rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone regimen on day 26, and she was discharged on day 45.
血管内大B细胞淋巴瘤(IVLBCL)是一种罕见的非霍奇金B细胞淋巴瘤,主要发生在毛细血管和小血管中。IVLBCL的成功诊断具有挑战性,因为它缺乏肿瘤形成且临床表现多样。一名82岁的亚洲女性患者因全身乏力、体重减轻和发热两周的病史前来我院急诊科就诊。患者的随机皮肤活检结果为阴性,骨髓活检显示噬血细胞综合征,未见明显淋巴瘤细胞浸润。镓扫描显示子宫、骨盆和脊柱有轻度摄取。重复骨髓活检结果及子宫内膜细胞学/活检均为阴性;然而,盆腔MRI结果与淋巴瘤相符,显示子宫体、骨盆和椎体有病变。在腹腔镜辅助下经阴道全子宫切除术及双侧输卵管卵巢切除术后,确诊为亚洲型IVLBCL。尽管对于身体状况下降的老年患者,全子宫切除术仍存在争议,但我们成功诊断了病情并开始了治疗。在第26天开始使用利妥昔单抗、环磷酰胺、阿霉素、长春新碱和泼尼松龙方案后,患者的一般状况很快得到改善,并于第45天出院。