Lymphoid blast crisis of B-lineage phenotype with monosomy 7 in a patient with juvenile chronic myelogenous leukemia (JCML).
Lymphoid blast crisis of B-lineage phenotype with monosomy 7 in a patient with juvenile chronic myelogenous leukemia (JCML).
复制标题
幼年型慢性粒细胞白血病 (JCML) 患者中具有 7 号单体的 B 谱系表型的淋巴母细胞危机。
作者：
Lau Rc;J. Squire;L. Brisson;S. Kamel‐Reid;T. Grunberger;I. Dubé;M. Letarte;K. Shannon;M. Freedman
我们研究了一个幼年型慢性粒细胞白血病（JCML）患者，其终末病程的特点是转化为急性淋巴细胞白血病。核型研究确定单体7白血病骨髓单核细胞的骨髓细胞在慢性期和淋巴母细胞在转化阶段。我们在培养中维持转化的淋巴母细胞的能力使我们能够进一步表征它们。存在CD 19、HLA-DR和CD 10，与前B急性淋巴细胞白血病表型一致。CD 14（My-4）和CD 13（My-7）均为阴性。免疫球蛋白重链和轻链基因的重排鉴定了B谱系细胞的单克隆群体。该病例进一步证明了JCML是一种多能干细胞起源的克隆性疾病。
We studied a patient with juvenile chronic myelogenous leukemia (JCML) whose terminal course was characterized by transformation to acute lymphoblastic leukemia. Karyotypic studies identified monosomy 7 in leukemic myelomonocytic marrow cells during the chronic phase and in the lymphoblasts during the transformation phase. Our ability to sustain the transformed lymphoblasts in culture allowed us to characterize them further. CD19, HLA-DR, and CD10 were present, consistent with a pre-B acute lymphoblastic leukemia phenotype. CD14 (My-4) and CD13 (My-7) were negative. Rearrangement of immunoglobulin heavy- and light-chain genes identified monoclonal populations of cells of the B lineage. This case provides further evidence that JCML is a clonal disease of pluripotent stem-cell origin.