Molica S, Brugiatelli M, Callea V, Morabito F, Levato D, Nobile F, Alberti A
Divisione di Ematologia, Ospedale Regionale A. Pugliese, Catanzaro, Italy.
Eur J Haematol. 1994 Apr;52(4):216-21. doi: 10.1111/j.1600-0609.1994.tb00648.x.
Fifty-three patients affected with B-cell chronic lymphocytic leukemia (CLL) younger than 50 years and observed in two hematological institutions have been retrospectively evaluated in order to verify whether this disease has different clinico-hematological features at presentation and different prognosis as compared to older cases. In our experience young cases with B-CLL diagnosis, confirmed by immunophenotype in 90.5% of patients, accounted for 7.1% of the whole CLL population. Sex distribution, mean peripheral lymphocyte count, platelet count, distribution among Rai's and Binet's stages, total tumor mass (TTM) score, histological pattern of bone marrow infiltration and lymphocyte doubling time (LDT) were similar to a series of 201 CLL cases older than 50 years. Only hemoglobin mean level was significantly higher in younger patients (13.1 +/- 2.1 vs 12.2 +/- 2.6 g/dl; p < 0.01). The overall median survival was 7.1 years. Rai and Binet staging classifications and TTM score system retained their prognostic value in this CLL population. In addition, cases fulfilling criteria of "smoldering" CLL, had a very long survival (75% survival probability at 16 years). Life-expectancy of younger patients was significantly longer than that of older ones (median survival, 7.1 versus 4.1 years; p < 0.05). However, when the background mortality due to non-CLL related deaths (i.e., cardiovascular complications, epithelial cancers) was removed, survival advantage of young cases disappeared. In conclusion this study confirms that prognosis of young CLL patients can be easily assessed using the current well-defined criteria. Since age is not by itself a criterion for intensifying treatment, further efforts to identify those young CLL patients who qualify for more aggressive therapy should be made.
为了验证50岁以下的B细胞慢性淋巴细胞白血病(CLL)患者与老年患者相比在疾病初发时是否具有不同的临床血液学特征及不同的预后,我们对在两家血液学机构观察到的53例此类患者进行了回顾性评估。根据我们的经验,经免疫表型确诊的B-CLL年轻患者占整个CLL患者群体的7.1%,其中90.5%的患者免疫表型得到确认。年轻患者的性别分布、外周血淋巴细胞平均计数、血小板计数、Rai分期和Binet分期分布、总肿瘤负荷(TTM)评分、骨髓浸润的组织学模式以及淋巴细胞倍增时间(LDT)与201例50岁以上的CLL患者相似。仅年轻患者的血红蛋白平均水平显著更高(13.1±2.1 vs 12.2±2.6 g/dl;p<0.01)。总体中位生存期为7.1年。Rai和Binet分期分类以及TTM评分系统在该CLL患者群体中保留了其预后价值。此外,符合“冒烟型”CLL标准的患者生存期非常长(16年时生存概率为75%)。年轻患者的预期寿命显著长于老年患者(中位生存期分别为7.1年和4.1年;p<0.05)。然而,去除非CLL相关死亡(即心血管并发症、上皮癌)导致的背景死亡率后,年轻患者的生存优势消失。总之,本研究证实,使用当前明确的标准可以轻松评估年轻CLL患者的预后。由于年龄本身并非强化治疗的标准,应进一步努力确定那些符合更积极治疗条件的年轻CLL患者。