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儿童急性淋巴细胞白血病形态学特征的预后意义

The prognostic significance of morphological features in childhood acute lymphoblastic leukaemia.

作者信息

Hann I M, Evans D I, Palmer M K, Morris-Jones P J, Haworth C

出版信息

Clin Lab Haematol. 1979;1(3):215-26. doi: 10.1111/j.1365-2257.1979.tb00470.x.

Abstract

A consecutive series of 209 children with acute lymphoblastic leukaemia (ALL) presenting to a regional referral unit between 1970 and 1977 was studied. The following morphological features in the initial bone marrow were recorded: blast size, percentage periodic acid-Schiff (PAS) and oil-red-O (ORO) positivity, percentage of blasts with vacuoles, and acid phosphatase positivity. The blasts were also coded according to the FAB (L13) classification. When analysed separately, increasing blast size was significantly related to the length of first remission (P = 0.01). However, this was almost entirely due to its association with the FAB L2 type of disease which also had a highly significant influence on length of first remission (P less than 0.0001) independent of all other factors. Patients with L3 disease had blasts with heavy vacuolation, ORO positivity and displayed monoclonal surface immunoglobulin and their prognosis was very poor. The percentage of vacuolated blasts and ORO positivity otherwise showed no influence on prognosis. Fine granularity of PAS staining in lymphoblasts showed no prognostic value but the trend of longer remission duration with increase in percentage of PAS coarse granularity and blocks, was statistically significant (P = 0.006). This relationship was partly due to a correlation with the presence of mediastinal mass and L2 disease, but was still independent of all other prognostic factors. In a smaller number (39) of the series of patients on whom cell surface markers were available, we were unable to demonstrate a correlation between T-derived blasts and the L1 and L2 classification; but there was a strong correlation with polar acid phosphatase positivity in T-derived blasts.

摘要

对1970年至1977年间在一个地区转诊单位就诊的209例急性淋巴细胞白血病(ALL)患儿进行了连续研究。记录了初始骨髓中的以下形态学特征:原始细胞大小、过碘酸希夫(PAS)和油红O(ORO)阳性百分比、有液泡的原始细胞百分比以及酸性磷酸酶阳性。原始细胞也根据FAB(L13)分类进行编码。单独分析时,原始细胞大小增加与首次缓解期长度显著相关(P = 0.01)。然而,这几乎完全归因于其与FAB L2型疾病的关联,该型疾病对首次缓解期长度也有高度显著影响(P小于0.0001),且独立于所有其他因素。L3疾病患者的原始细胞有大量液泡、ORO阳性,并显示单克隆表面免疫球蛋白,其预后很差。液泡化原始细胞百分比和ORO阳性在其他方面对预后无影响。淋巴母细胞中PAS染色的细颗粒度无预后价值,但随着PAS粗颗粒度和块状物百分比增加,缓解持续时间延长的趋势具有统计学意义(P = 0.006)。这种关系部分归因于与纵隔肿块和L2疾病存在的相关性,但仍独立于所有其他预后因素。在该系列中少数(39例)有细胞表面标志物的患者中,我们未能证明T来源的原始细胞与L1和L2分类之间存在相关性;但T来源的原始细胞与极性酸性磷酸酶阳性之间存在强相关性。

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