Ineffective erythropoiesis in the spleen of a patient with pyruvate kinase deficiency
Ineffective erythropoiesis in the spleen of a patient with pyruvate kinase deficiency
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DOI：
10.1002/ajh.10380
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发表时间：
2003-09-01
影响因子：
12.8
通讯作者：
Kanno, H
中科院分区：
文献类型：
--
作者：
Aizawa, S;Kohdera, U;Kanno, H
在这项研究中，丙酮酸激酶（PK）缺乏对红系祖细胞成熟的可能的不利影响进行了调查。一名患有严重PK缺乏症的4岁日本女孩接受了脾切除术，以减少输血需求。对脾脏进行组织学检查，并测定脾脏中的造血祖细胞以评估该PK缺陷受试者的髓外造血。PK缺陷患者脾脏中的造血祖细胞包括CFU-GM、BFU-E和CFU-GEMM的数量远高于对照脾脏中发现的那些，表明髓外造血增强。原位末端标记法（TUNEL）检测显示PK缺陷患者的脾红髓中存在凋亡细胞。7A 6抗原在脾细胞和体外培养的细胞中均有表达，但仅在血型糖蛋白A阳性的细胞中有表达。这些结果提供了PK缺乏中的代谢紊乱不仅影响红细胞的存活而且影响红系祖细胞的成熟的证据，这导致过早的细胞死亡，即，凋亡(C)2003 Wiley-Liss，Inc.
In this study, possible adverse effects of pyruvate kinase (PK) deficiency on the maturation of erythroid progenitors were investigated. A 4-year-old Japanese girl with severe PK deficiency underwent splenectomy to reduce her need for blood transfusions. The spleen was examined a histologically, and the hematopoietic progenitors in the spleen were assayed to evaluate the extramedullary hematopoiesis of this PK-deficient subject. The number of hematopoietic progenitors including CFU-GM, BFU-E and CFU-GEMM in the spleen of the PK-deficient patient was much higher than those found in control spleens, indicating enhanced extramedullary hematopoiesis. TUNEL assay demonstrated apoptotic cells in the splenic red pulp of the PK-deficient patient. The expression of 7A6 antigen was detected in cells isolated from spleen and in cells cultured in vitro, but only in those cells that were positive for glycophorin A. These results provide evidence that the metabolic disturbances in PK deficiency affect not only the survival of red cells but also the maturation of erythroid progenitors, which results in premature cell death, i.e., apoptosis. (C) 2003 Wiley-Liss, Inc.