Nikitin E A, Lorie Iu Iu, Melikian A L, Samoĭlova R S, Bulycheva T I, Obukhova T N, Kaplanskaia I B, Doronin V A, Kolosova L Iu, Goriacheva S R
Ter Arkh. 2003;75(7):38-47.
To assess factors of an unfavourable prognosis in a group of intermediate risk of B-cell chronic lymphoid leukemia (BCCLL).
206 BCCLL patients (mean age 55.5 years, male/female = 1.66) entered the study conducted by Hematological Research Center in 1992-2000.
Nine patients under 35 years of age did not survive 5 years except one female who achieved a complete remission on fludarabin. The type of bone marrow infiltration (diffuse vs interstitial and nodular), the time of lymphocyte count doubling (under or over 12 months) discriminate the patients by prognosis in the group of intermediate risk: medians of overall survival 65 months vs 148 months and 72 vs 133 months, respectively (p < 0.005 for both curves, log-rank criterion). Survival medians in groups with low (< 50% cells) and high (> 50% cells) expression of CD38+ cells in the group of intermediate BCCLL risk comprise 55 and 106 months (p = 0.005). The type of bone marrow infiltration and time of doubling of lymphocyte count overlap: > 70% patients with a diffuse type of bone marrow infiltration have the time of doubling under 12 months and vice versa while expression of CD38 do not overlap with these values. Combination of two signs (type of bone marrow infiltration and CD38 expression or time og lymphocyte count doubling and CD38 expression) allows more precise identification of prognostically unfavourable groups. Medians of survival for combination of the first two signs (two positive against two negative) comprise 51 months vs 169 months (p < 0.0001), for combination of the latter two signs 55 months vs 106 months was not reached (p < 0.001). Although most patients with a tumor form of BCCLL are referred to stage II, the prognosis in this form is much worse than in stage II, survival medians are 44 and 69 months, respectively (p < 0.05). A mutation status of the genes of a variable region of immunoglobulins enable identification of the group of patients with a relatively benign course of BCCLL (survival medians 61 and 289 months, p < 0.0001).
In patients under 35 years of age BCCLL runs unfavourably and seems to require intensive polychemotherapy. Usage of a combination of the signs (CD38, time of doubling of lymphocyte count and type of bone marrow infiltration) is a simple and reliable method of identification of prognostically different categories of patients in the group of an intermediate BCCLL risk. Prognosis in patients with a tumor form of BCCLL is unfavourable: medians of survival in patients with a tumor form and stage III-IV are comparable. Mutational status of the genes of immunoglobulin variable region may serve a marker of a long-term prognosis.
评估一组处于B细胞慢性淋巴细胞白血病（BCCLL）中危风险患者预后不良的因素。
206例BCCLL患者（平均年龄55.5岁，男/女 = 1.66）于1992年至2000年进入血液学研究中心开展的研究。
9例35岁以下患者中除1例接受氟达拉滨治疗后完全缓解的女性外，均未存活5年。骨髓浸润类型（弥漫性与间质结节性）、淋巴细胞计数倍增时间（12个月以下或以上）在中危组患者中可根据预后进行区分：总生存中位数分别为65个月对148个月以及72个月对133个月（两条曲线的p值均 < 0.005，对数秩检验标准）。中危BCCLL组中CD38 +细胞低表达（< 50%细胞）和高表达（> 50%细胞）组的生存中位数分别为55个月和106个月（p = 0.005）。骨髓浸润类型和淋巴细胞计数倍增时间存在重叠：> 70%骨髓浸润为弥漫性的患者其倍增时间在12个月以下，反之亦然，而CD38表达与这些值无重叠。两种指标的联合（骨髓浸润类型和CD38表达或淋巴细胞计数倍增时间和CD38表达）可更精确地识别预后不良组。前两种指标联合（两个阳性对两个阴性）的生存中位数为51个月对169个月（p < 0.0001），后两种指标联合为55个月对106个月（未达到统计学差异，p < 0.001）。尽管大多数肿瘤型BCCLL患者被归为II期，但这种类型的预后比II期差得多，生存中位数分别为44个月和69个月（p < 0.05）。免疫球蛋白可变区基因突变状态能够识别出BCCLL病程相对良性的患者组（生存中位数为61个月和289个月，p < 0.0001）。
35岁以下的BCCLL患者预后不佳，似乎需要强化多药化疗。联合使用多种指标（CD38、淋巴细胞计数倍增时间和骨髓浸润类型）是一种简单可靠的方法，可用于识别中危BCCLL组中预后不同的患者类别。肿瘤型BCCLL患者的预后不佳：肿瘤型患者与III - IV期患者的生存中位数相当。免疫球蛋白可变区基因的突变状态可作为长期预后的标志物。
