Baranger L, Baruchel A, Leverger G, Schaison G, Berger R
Unité INSERM U 301 et SDI No. 15954 CNRS, Institute de Génétique Moléculaire, Paris, France.
Leukemia. 1990 May;4(5):345-9.
Acquired pure monosomy-7 is associated with various myeloproliferative disorders (MPD), myelodysplasias (MDS), and acute myeloblastic leukemias (AML) in children and a poor prognosis. A series of 14 malignant blood disorders with pure monosomy-7 in children (eight MPD, two refractory anemia with excess of blasts, (RAEB), and four AML) is reported and compared with cases in the literature. The median age is significantly different in the patients with MPD and those with MDS or AML: 23, 80.5, and 112 months, respectively. The outcomes of MPD and RAEB are characterized by a high risk of rapid blastic transformation and resistance to polychemotherapy. Bone marrow transplantation (BMT) seems to be the best treatment, and one survival of two years in complete remission after autologous BMT in a child with AML is reported. Several myeloid cell lineages are involved in the proliferation, which partly explains the difficulties of cytologic classification and suggests that a pluripotent stem-cell is at the origin of the disease.
获得性纯单体7与儿童的各种骨髓增殖性疾病（MPD）、骨髓发育异常（MDS）和急性髓细胞白血病（AML）相关，且预后不良。本文报告了一系列14例儿童患有纯单体7的恶性血液疾病（8例MPD、2例伴有原始细胞增多的难治性贫血（RAEB）和4例AML），并与文献中的病例进行了比较。MPD患者与MDS或AML患者的中位年龄有显著差异：分别为23、80.5和112个月。MPD和RAEB的结局特点是快速原始细胞转化风险高且对多药化疗耐药。骨髓移植（BMT）似乎是最佳治疗方法，据报道，1例AML儿童患者在自体BMT后两年完全缓解存活。几种髓系细胞谱系参与增殖，这部分解释了细胞学分类的困难，并提示多能干细胞是该疾病的起源。
