Juvenile chronic myelogenous leukaemia: the only example of truly fetal (not fetal-like) erythropoiesis.
Juvenile chronic myelogenous leukaemia: the only example of truly fetal (not fetal-like) erythropoiesis.
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青少年慢性粒细胞白血病：真正胎儿（非胎儿样）红细胞生成的唯一例子。
DOI：
10.1111/j.1365-2141.1990.tb07891.x
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发表时间：
1990
影响因子：
6.5
通讯作者：
Alter,BP
中科院分区：
文献类型：
--
作者：
Weinberg,RS;Leibowitz,D;Weinblatt,ME;Kochen,J;Alter,BP
308例病例报告脱水酶低，i抗原滴度高（Weatherall等人，1968; Maurer等人，1972）。我们以前曾报道，在血红蛋白病患者和骨髓移植恢复期患者中观察到的HbF水平升高发生在成人“胎儿样”而不是真正的胎儿祖细胞的后代中（温伯格等人，1983，1984，1986）。这些祖细胞可以在红系培养物中区分。在甲基纤维素培养物中，胎儿红系祖细胞早期发育成红系爆发形成单位（BFU-E）衍生的集落。这些菌落含有高水平的HbF，主要由γ球蛋白链组成。γ和总γ珠蛋白的相对合成在新生BFU-E衍生的集落中是相关的。相比之下，成年“胎儿样”祖细胞分化成合成较少HbF的细胞，具有较低比例的HbF。戈伊和总γ球蛋白的合成在个体成人“胎儿样”BFU-E衍生的集落中不相关，这些集落稍后出现在培养物中。在这里介绍的研究中，红细胞培养表明，我们病人的异常祖细胞是真正的胎儿起源。因此，JCML是唯一真正胎儿红细胞生成的产后例子。该患者是一名7岁男孩，表现为苍白、贫血、血小板减少和肝脾肿大。他最初的血细胞计数是：红细胞压积24.1%，血小板计数17 x 10 y/l，白色血细胞计数24.2 x 10 y/l，粒细胞38%，淋巴细胞22%，单核细胞14%，有核红细胞22%。他的骨髓表现为红系增生伴巨核细胞减少、红细胞吞噬作用，无恶性或原始血液细胞。一年多后，HbF上升到73%。白细胞碱性磷酸酶异常低下
308 Case Reports anhydrase is low, and i antigen titres are high (Weatherall et al, 1968; Maurer et al, 1972). We have previously reported that elevated HbF levels observed in patients with haemoglobinopathies and patients recovering from bone marrow transplantation, occur in the progeny of adult'fetal-like'rather than truly fetal progenitors (Weinberg et al, 1983, 1984, 1986). These progenitors can be distinguished in erythroid cultures. Fetal erythroid progenitors develop into burst forming unit-erythroid (BFU-E)-derived colonies early in methyl cellulose cultures. These colonies contain high levels of HbF composed mostly of" y globin chains. The relative syntheses of': y and total y globin are correlated in newborn BFU-E-derived colonies. In contrast, adult'fetal-like'progenitors differentiate into cells which synthesize less HbF with a lower proportion of". The syntheses of Gy and total y globin are not correlated in individual adult'fetal-like'BFU-E-derived colonies, which appear later in culture. In the studies presented here, erythroid cultures indicated that the abnormal progenitor cells from our patient were of truly fetal origin. Thus, JCML is the only postnatal example of truly fetal erythropoiesis. The patient was a 7-year-old boy who presented with pallor, anaemia, thrombocytopenia and hepatosplenomegaly. His initial blood count was: haematocrit 24.1%, platelet count 17 x 10y/l, and white blood cell count 24.2 x 10y/l, with 38% granulocytes, 22% lymphocytes, 14% monocytes and 22% nucleated red blood cells. His presentation bone marrow had erythroid hyperplasia with decreased megakaryocytes, erythrophagocytosis, and no malignant or primitive haematologic cells. More than a year later, HbF had risen to 73%. leucocyte alkaline phosphatase was abnormally low