IgD型浆母细胞骨髓瘤一例报告并文献复习

IgD Plasmablastic Plasma Cell Myeloma: A Case Report and Literature Review

摘要: 目的：探讨浆母细胞骨髓瘤患者的临床特征、诊治和预后。方法：报道1例IgD型浆母细胞骨髓瘤患者的诊疗，结合文献复习，探讨该疾病的发病机制、诊断、治疗与预后特征。结果：患者，男，53岁，确诊为IgD型浆母细胞骨髓瘤。给予包含硼替佐米在内的多个疗程的化疗后，疾病仍然进展。患者因肿瘤累及椎管行肿瘤切除术，腰椎肿物病理示浆细胞骨髓瘤(浆母细胞型)，术后3个月内死亡，总生存期：18个月。结论：IgD型和浆母细胞骨髓瘤均较罕见，二者存活时间短，预后差。浆母细胞骨髓瘤病因尚不清楚，临床表现无特异性，确诊主要依靠病理及免疫组织化学检查，目前尚无标准的治疗方案，多用类似于多发性骨髓瘤治疗的方案，包括新药在内的高强度化疗以及自体造血干细胞移植可能疗效更好。

Abstract: Objective: To investigate the clinical features, diagnosis, therapy and prognosis of IgD plasmablastic plasma cell myeloma. Methods: The diagnosis and therapy of a patient with IgD plasmablastic plasma cell myeloma were reported, and the pathogenesis, diagnosis, therapy and prognosis of the disease were discussed in combination with literature review. Results: The patient, a 53-year-old man, was diagnosed with IgD plasmablastic plasma cell myeloma. Progressive disease remains after multiple courses of chemotherapy including bortezomib. The patient underwent tumor resection due to tumor involving the spinal canal, pathology of the lumbar mass showed plasmablastic plasma cell myeloma, and the patient died within 3 months after surgery, with an overall survival of 18 months. Conclusion: IgD and plasmablastic plasma cell myelomas were extremely rare. These patients usually had a poor prognosis. Its pathogenesis remained to be unclear. With non-specific clinical manifestations, the diagnosis was mainly confirmed by histopathological and immunohistochemical staining method. Without standard treatment, these patients were treated with chemotherapy regimens, including new drugs, high-intensity chemotherapy and autologous hematopoietic stem cells.