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人类白血病前期研究。IV. 骨髓细胞增多的再生障碍性贫血中环形铁粒幼细胞的临床及预后意义。

Studies on human preleukaemia. IV. Clinical and prognostic significance of sideroblasts in aregenerative anaemia with hypercellular bone marrow.

作者信息

Hast R

出版信息

Scand J Haematol. 1978 Nov;21(5):396-402.

PMID:734352
Abstract

The prognostic significance of the bone marrow sideroblasts found in aregenerative anaemia with hypercellular bone marrow was evaluated particularly to see if the sideroblasts might be a preleukaemic sign. The study includes 32 patients with abnormal sideroblasts; 14 had mainly ringed sideroblasts while 18 had mainly the intermediate or ferritin type. Basic erythrokinetic data (59Fe erythrocyte incorporation and erythroblast labelling indices) were similar in the two groups. The colony forming capacity (CFUc) was abnormal in 2 of 5 patients with ringed sideroblasts compared to 12 of 13 in the intermediate sideroblast group. Patients with mainly ringed sideroblasts had significantly longer duration of disease state, higher platelet and leucocyte counts, no increase in the percentage of bone marrow myeloblasts and did less often develop acute leukaemia. Thus aregenerative anaemias can be divided according to the type of abnormal sideroblasts in two groups with different risk of development of acute leukaemia. The ringed sideroblastic anaemia seems not to be a preleukaemic stage in the prospective sense in contrast to aregenerative anaemia with mainly intermediate sideroblasts.

摘要

对再生障碍性贫血伴骨髓细胞增多时发现的骨髓环形铁粒幼细胞的预后意义进行了评估,尤其旨在观察环形铁粒幼细胞是否可能为白血病前期征象。该研究纳入了32例异常铁粒幼细胞患者;14例主要为环形铁粒幼细胞,18例主要为中间型或铁蛋白型。两组的基础红细胞动力学数据(59Fe红细胞摄取和幼红细胞标记指数)相似。环形铁粒幼细胞组5例患者中有2例集落形成能力(CFUc)异常,而中间型铁粒幼细胞组13例中有12例异常。主要为环形铁粒幼细胞的患者疾病状态持续时间显著更长,血小板和白细胞计数更高,骨髓原粒细胞百分比无增加,发生急性白血病的情况也较少。因此,再生障碍性贫血可根据异常铁粒幼细胞的类型分为两组,其发生急性白血病的风险不同。与主要为中间型铁粒幼细胞的再生障碍性贫血相反,环形铁粒幼细胞性贫血从前瞻性角度看似乎并非白血病前期阶段。

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