Gertz Morie A
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Leuk Lymphoma. 2002 Aug;43(8):1517-26. doi: 10.1080/1042819021000002839.
Waldenstrom's macroglobulinemia is a lymphoplasmacytic disorder characterized by a monoclonal IgM protein, anemia, hepatosplenomegaly, and hyperviscosity. With the increasing use of screening chemistry evaluations, many patients are diagnosed without symptoms and are candidates for observation with no therapeutic intervention until symptoms develop. Plasma exchange can be useful to manage hyperviscosity but does not address the infiltrative process in the bone marrow, which requires cytoreductive therapy. This review covers current regimens that have been used to manage Waldenstrom's macroglobulinemia, including alkylating agents, purine nucleoside analogs, and rituximab. The value of steroids, radiotherapy, stem cell transplantation, and splenectomy is also reviewed. The lack of phase III studies does not permit an algorithm that would be appropriate for all patients. Treatment needs to be individualized based on patient age, the clinical manifestations of Waldenström's, and the patient's potential for developing toxic side effects of the selected treatment regimen.
华氏巨球蛋白血症是一种淋巴浆细胞性疾病，其特征为单克隆IgM蛋白、贫血、肝脾肿大和血液黏稠度增加。随着筛查化学评估的使用日益增多，许多患者在无症状时被诊断出来，成为观察对象，在症状出现之前无需进行治疗干预。血浆置换有助于处理血液黏稠度问题，但无法解决骨髓中的浸润过程，而这需要进行减瘤治疗。本综述涵盖了目前用于治疗华氏巨球蛋白血症的方案，包括烷化剂、嘌呤核苷类似物和利妥昔单抗。还综述了类固醇、放射治疗、干细胞移植和脾切除术的价值。缺乏III期研究使得无法制定适用于所有患者的治疗方案。治疗需要根据患者年龄、华氏巨球蛋白血症的临床表现以及患者对所选治疗方案产生毒副作用的可能性进行个体化。
