QUANTITATIVE-EVALUATION OF ERYTHROPOIETIC ACTIVITY IN DYSMYELOPOIETIC SYNDROMES
QUANTITATIVE-EVALUATION OF ERYTHROPOIETIC ACTIVITY IN DYSMYELOPOIETIC SYNDROMES
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DOI：
10.1111/j.1365-2141.1982.tb01890.x
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发表时间：
1982-01-01
影响因子：
6.5
通讯作者：
ASCARI, E
中科院分区：
文献类型：
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作者：
CAZZOLA, M;BAROSI, G;ASCARI, E
根据骨髓和外周血形态学表现，将43例骨髓造血障碍综合征分为4型：原始细胞增多型难治性贫血、慢性粒单核细胞白血病、原发性获得性铁粒幼细胞性贫血和骨髓有细胞型难治性贫血，但无原始细胞和/或环形铁粒幼细胞。铁动力学允许定义3个不同的患者组。所有难治性贫血伴原始细胞增多和慢性粒单核细胞白血病病例均归为同一组。他们的特点是相对骨髓衰竭，并有很高的可能性发展为急性白血病。另一方面，患有原发性获得性铁粒幼细胞性贫血的个体具有高的红细胞生成活性，但基本上无效。他们有一个良性的临床过程没有证据表明白血病转化。在中间组中，在红细胞生成活性方面，有难治性贫血的非专业患者，有细胞性骨髓和少数原发性获得性铁粒幼细胞性贫血的个体。他们的临床病程和发生急性白血病的风险介于其他两组之间。不同的实体可能存在于骨髓增生异常综合征的范围内。在临床实践中，它们可以通过形态学研究和其他简单的实验室手段来识别。
Based on the morphological appearances of the bone marrow and peripheral blood, 43 patients with dysmyelopoietic syndromes were categorized into 4 types: refractory anemia with excess of blasts, chronic myelomonocytic leukemia, primary acquired sideroblastic anemia and refractory anemia with cellular marrow, without excess of blasts and/or ring sideroblasts. Ferrokinetics allowed 3 distinct groups of patients to be defined. All cases of refractory anemia with excess of blasts and chronic myelomonocytic leukemia were classified in the same group. They were characterized by relative marrow failure and had a high likelihood of developing acute leukemia. At the other end of the spectrum, individuals with primary acquired sideroblastic anemia had high erythropoietic activity which was largely ineffective. They had a benign clinical course without evidence of leukemic transformation. In the middle group, in terms of erythropoietic activity, lay patients with refractory anemia with cellular marrow and a few individuals with primary acquired sideroblastic anemia. Their clinical course and the risk of developing acute leukemia were intermediate between the other 2 groups. Separate entities may exist within the spectrum of dysmyelopoietic syndromes. In clinical practice, they may be recognized by morphological studies and other simple laboratory means.