Chronic myelogenous leukemia: recent advances.

Chronic myelogenous leukemia: recent advances.
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DOI:
10.1182/blood.v65.5.1039.bloodjournal6551039
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发表时间:
1985-05
期刊:
影响因子:
20.3
通讯作者:
Richard E. Champlin;David W. Golde
Richard E. Champlin;David W. Golde
中科院分区:
医学1区
文献类型:
--
作者:
Richard E. Champlin;David W. Golde

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通过R.E. Champlin和OW。慢性粒细胞性白血病(CML)是一种血液恶性肿瘤,其特征是髓系细胞及其祖细胞过度生长。[2] 1845年,Craigie [3]班尼特[4]和Virchow [#{176}]首次描述了这种疾病,他们注意到血液“化脓”和显著的脾肿大的典型特征。CML是第一个与一致的染色体异常相关的人类肿瘤,费城染色体(Ph '),其存在于超过90%的病例中。6细胞遗传学研究和葡萄糖-6-磷酸脱氢酶(G6 PD)同工酶和分析7' 8已经证明CML是多能造血干细胞的克隆性疾病。在粒细胞、单核细胞、巨噬细胞、红细胞、巨核细胞、嗜酸性粒细胞、嗜碱性粒细胞和它们的混合祖细胞中鉴定出Ph'染色体或G6 PD同工酶表达的单克隆模式。9'混合集落形成细胞CFU-GEMM也含有Ph'染色体。' 4 B淋巴细胞和一些无效细胞也来自肿瘤克隆。目前还不清楚T淋巴细胞参与恶性过程的程度。尚未报道对凝集素应答的外周血T细胞含有Ph'染色体,并且通过G6 PD分析是多克隆的。7然而,有可能存在少量的Ph '阳性T细胞,但在大量正常长寿T淋巴细胞中无法检测到。最近,已经报道了几例T淋巴细胞原始细胞危象,这表明该疾病可能涉及能够分化为T淋巴细胞以及其他骨髓和淋巴细胞的多能细胞。8“9 Ph'染色体不存在于骨髓成纤维细胞和其他间充质组织中。
By R.E. Champlin and OW. Golde C HRONIC myelogenous leukemia (CML) is a hematologic malignancy characterized by excessive growth of myeloid cells and their progenitors.”2 The disease was originally described in 1845 by Craigie,3 Bennett,4 and Virchow,#{176}who noted the classic features of “purulence” of the blood and marked splenomegaly. CML was the first human neoplasm to be associated with a consistent chromosome abnormality, the Philadelphia chromosome (Ph’), which is present in over 90% of cases.6 Cytogenetic studies and glucose-6-phosphate dehydrogenase (G6PD) isoenzyme and analyses7’8 have demonstrated that CML is a clonal disorder of pluripotent hematopoietic stem cells. The Ph’ chromosome or a monoclonal pattern of G6PD isoenzyme expression has been identified in granulocytes, monocytes, macrophages, erythrocytes, megakaryocytes, eosinophils, basophils, and their cornmitted progenitors.9 ‘ Mixed colony-forming cells, CFU-GEMM, also contain the Ph’ chromosome.’4 B lymphocytes and some null cells are also derived from the neoplastic clone.’5”6 It is unclear to what extent T lymphocytes are involved in the malignant process. Peripheral blood T cells responding to lectin have not been reported to contain the Ph’ chromosome and are polyclonal by G6PD analysis.’7 It is possible, however, that small numbers of Ph’-positive T cells are present but cannot be detected among a predominance of normal long-lived T lymphocytes. Recently, several cases of T lymphocyte blast crisis have been reported, suggesting that the disease may involve a pluripotent cell capable of differentiating to T lymphocytes as well as to other myeloid and lymphoid cells.’8”9 The Ph’ chromosome is not present in bone marrow fibroblasts and other mesenchymal tissues.2#{176}