Takaya N, Miyakoshi S, Kubo K, Mutou Y, Endou Y
Department of Hematology, Toranomon Hospital.
Rinsho Ketsueki. 1998 May;39(5):379-85.
A 62-year-old woman with IgA-lambda type monoclonal gammopathy had been followed up since January 1988. In March 1991, multiple myeloma (IgA-lambda) was diagnosed on the basis of bone marrow biopsy findings and increased serum IgA levels. She was treated intermittently with melphalan and prednisolone over a perioa of about 6 years, but was eventually admitted due to renal dysfunction, hypercalcemia, increased serum IgA and the formation of subcutaneous masses. During chemotherapy she underwent emergency surgery for obturative ileus. Histological examination of the resected tissues revealed invasion of myeloma cells into the small intestine and peritoneum. Despite continued chemotherapy, the patient's soft tissue masses enlarged, and new lesions appeared in other organs. In the terminal stage, lower serum IgA levels were observed despite an increase in Bence-Jones protein levels in urine. The patient died five months after admission. An autopsy found infiltration by atypical myeloma cells in multiple organs. An immunohistochemical examination revealed and increase in lambda-light chain positive cells relative to the number of alpha-heavy chain positive cells. The terminal course was considered to be representative of aggressive phase multiple myeloma. The case was rare in that the patient's ileus was caused by invasion of myeloma cells into the small intestine.
一名患有IgA-λ型单克隆丙种球蛋白病的62岁女性自1988年1月起接受随访。1991年3月,根据骨髓活检结果和血清IgA水平升高诊断为多发性骨髓瘤(IgA-λ型)。她在约6年的时间里间断接受美法仑和泼尼松龙治疗,但最终因肾功能不全、高钙血症、血清IgA升高及皮下肿块形成而入院。化疗期间,她因闭襻性肠梗阻接受了急诊手术。切除组织的组织学检查显示骨髓瘤细胞侵犯小肠和腹膜。尽管持续化疗,患者的软组织肿块仍增大,其他器官出现新病变。终末期,尽管尿本周氏蛋白水平升高,但血清IgA水平降低。患者入院5个月后死亡。尸检发现多个器官有非典型骨髓瘤细胞浸润。免疫组织化学检查显示,相对于α重链阳性细胞数量,λ轻链阳性细胞数量增加。终末期病程被认为是侵袭性多发性骨髓瘤的典型表现。该病例罕见之处在于患者的肠梗阻是由骨髓瘤细胞侵犯小肠所致。