Orii K, Kobayashi H, Ueno M, Ishida F, Saito H, Hata S, Aoki K, Narita A, Shimodaira S, Kitano K, Uchimaru K, Motokura T
Department of Internal Medicine, Nagano Red Cross Hospital.
Rinsho Ketsueki. 1997 Jun;38(6):520-5.
A 79-year-old male was admitted to our hospital because of general fatigue and night sweat. Physical examination showed generalized superficial lymphadenopathy, marked splenomegaly, and tumors in the conjunctiva and the abdomen. Chest X-ray and computed tomography (CT) revealed pleural effusion and intrathoracic lymphadenopathy. Abdominal ultrasonography and CT showed hepatosplenomegaly and intraperitoneal tumors. Upper gastrointestinal fiberscopy revealed multiple polypoid lesions and ulcers in the duodenum and the stomach. Involvement of relatively small-sized lymphocytes with cleaved nuclei was identified in each biopsied specimen from a cervical lymph node, a tumor in the conjunctiva, gastrointestinal polypoid lesions, and the bone marrow. Surface marker analysis of abnormal lymphocytes in the bone marrow revealed that CD5, CD19, and CD20 were strongly positive, but CD23 was weakly positive. Although (11:14)(q13:q32) translocation was not identified by chromosome analysis of bone marrow cells, Northern blot analysis of bone marrow cells revealed overexpression of the PRAD1 oncogene. Diagnosis of mantle cell lymphoma (MCL) was made. Combination chemotherapy by cyclophosphamide and vincristine was not effective, but etoposide perorally given at a dose of 50 mg per day was effective. In MCL, extranodal involvement of a digestive tract and bone marrow is well known. This case suggests that involvement of multiple organs including lacrimal glands and pleura could be characteristic of MCL cells.
一名79岁男性因全身乏力和盗汗入院。体格检查发现全身浅表淋巴结肿大、脾脏明显肿大,结膜和腹部有肿瘤。胸部X线和计算机断层扫描(CT)显示胸腔积液和胸内淋巴结肿大。腹部超声和CT显示肝脾肿大和腹腔内肿瘤。上消化道纤维内镜检查发现十二指肠和胃有多个息肉样病变和溃疡。从颈部淋巴结、结膜肿瘤、胃肠道息肉样病变和骨髓的每个活检标本中均发现有核裂的相对小淋巴细胞浸润。对骨髓中异常淋巴细胞的表面标志物分析显示,CD5、CD19和CD20呈强阳性,但CD23呈弱阳性。虽然通过骨髓细胞染色体分析未发现(11:14)(q13:q32)易位,但骨髓细胞的Northern印迹分析显示PRAD1癌基因过表达。诊断为套细胞淋巴瘤(MCL)。环磷酰胺和长春新碱联合化疗无效,但每天口服50 mg依托泊苷有效。在MCL中,消化道和骨髓的结外受累是众所周知的。该病例提示,包括泪腺和胸膜在内的多个器官受累可能是MCL细胞的特征。