Ahmed Mohammed, Al-Ghamdi Abdullah, Al-Omari Mohammed, Aljurf Mahmoud, Al-Kadhi Yusuf
Departments of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Ann Saudi Med. 2009 May-Jun;29(3):219-22. doi: 10.4103/0256-4947.51785.
Extramedullary adrenal plasmacytoma (EMP) involving the adrenal glands is rarely encountered clinically. We report a A 47-year-old male who presented with bilateral adrenal incidentalomas. After confirming EMP, the patient received two consecutive autologous hematopoietic stem cell transplants (HSCT) using high-dose melphalan. Following HSCT, a serial follow-up helical CT revealed a substantial decrease in the size of both adrenal masses. Serial periodic serum protein and urine electrophoresis and immunofixation showed abrogation of a previously noted monoclonal band. At 50 months follow-up the patient was alive and well. Our patient is the first with EMP to have received an autologous HSCT, which may prove to have a role in therapy due to the immunological effect of the infused donor marrow T-lymphocytes against the clonal proliferation of abnormal plasma cells in extrammedullary sites.This case indicates that an EMP should be added to the differential diagnosis of adrenal incidentalomas.
临床上很少遇到累及肾上腺的髓外肾上腺浆细胞瘤(EMP)。我们报告一例47岁男性,其表现为双侧肾上腺偶发瘤。确诊为EMP后,患者接受了两次连续的大剂量美法仑自体造血干细胞移植(HSCT)。HSCT后,系列随访螺旋CT显示双侧肾上腺肿块大小显著减小。系列定期血清蛋白和尿电泳及免疫固定显示先前发现的单克隆条带消失。随访50个月时,患者存活且状况良好。我们的患者是首例接受自体HSCT的EMP患者,由于输注的供体骨髓T淋巴细胞对髓外部位异常浆细胞克隆增殖的免疫作用,自体HSCT可能在治疗中发挥作用。该病例表明,肾上腺偶发瘤的鉴别诊断应增加EMP。