Bladé J, Kyle R A, Greipp P R
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Br J Haematol. 1996 May;93(2):345-51. doi: 10.1046/j.1365-2141.1996.5191061.x.
The purpose of this study was to analyse the presenting clinical and laboratory features and the outcome of 72 patients with multiple myeloma (MM) who were younger than 40 years. The records of all Mayo Clinic patients with MM younger than 40 years who were seen between 1 January 1956 and 31 December 1992 were reviewed. Survival was measured from the date when treatment was required to the date of last follow-up or death. The frequency of MM in patients younger than 40 and 30 years in 3278 Mayo Clinic patients was 2.2% and 0.3%, respectively. The main presenting clinical features were bone pain (66%), fatigue (26%), extramedullary plasmacytomas (19%) and bacterial infection (11%). Renal function impairment (creatinine level > or = 177 micromol/l) and hypercalcaemia (serum calcium value > or = 2.75 mmol/l) occurred in 29% and 30% of patients, respectively. Among the 57 patients evaluable for response the objective response rate was 54%. 14/35 patients treated with a single alkylating agent achieved an objective response, whereas 17/22 patients given combination chemotherapy had an objective response (P=0.013). However, this higher response rate did not result in a significantly longer survival. The median survival for the 72 patients was 54 months. Patients with good prognostic features (normal renal function or low beta 2-microglobulin level) had a median survival of 8 years. The actuarial survival at 5 and 10 years after initiation of therapy was 43% and 13%, respectively. In summary, survival in very young patients with myeloma is longer than that observed in series of patients of all ages, especially in those with good prognostic factors.
本研究旨在分析72例年龄小于40岁的多发性骨髓瘤(MM)患者的临床表现、实验室检查特征及预后情况。回顾了1956年1月1日至1992年12月31日期间梅奥诊所所有年龄小于40岁的MM患者的病历。从开始治疗到最后一次随访或死亡的日期计算生存期。在3278例梅奥诊所患者中,年龄小于40岁和30岁的MM患者发生率分别为2.2%和0.3%。主要临床表现为骨痛(66%)、疲劳(26%)、髓外浆细胞瘤(19%)和细菌感染(11%)。分别有29%和30%的患者出现肾功能损害(肌酐水平≥177μmol/L)和高钙血症(血清钙值≥2.75mmol/L)。在57例可评估缓解情况的患者中,客观缓解率为54%。35例接受单一烷化剂治疗的患者中有14例获得客观缓解,而22例接受联合化疗的患者中有17例获得客观缓解(P=0.013)。然而,这种较高的缓解率并未导致生存期显著延长。72例患者的中位生存期为54个月。具有良好预后特征(肾功能正常或β2-微球蛋白水平低)的患者中位生存期为8年。开始治疗后5年和10年的精算生存率分别为43%和13%。总之,非常年轻的骨髓瘤患者的生存期比所有年龄段患者系列中观察到的生存期更长,尤其是那些具有良好预后因素的患者。