177例慢性粒细胞白血病患者血糖测定值分析

目的 探讨可能影响慢性粒细胞白血病（CML）患者血糖测定值的因素，加深对白血病假性低血糖现象的认识。方法 应用自动生化分析仪对177例CML患者血糖和外周血白细胞进行测定，并结合临床进行分析。结果 在177例CML患者中，共发现32例（18.1%)白血病假性低血糖现象。血糖测定值与白细胞计数呈负相关（P＜0.05)，环境温度对假性低血糖现象的发生也有一定的影响。32例白血病假性低血糖现象中，24例未得到临床医师的充分认识。此外，5例（2.8%)CML急变合并高血糖患者，其原因可能是应激导致了血糖增高。结论 外周血白细胞计数和环境温度对白血病假性低血糖现象的产生有一定作用，对CML血糖值的分析应与临床症状相结合。     

Missing Y chromosome in Ph1-negative chronic myeloid leukemia with bcr rearrangement. Evidence for a bcr-abl recombination on chromosome 22 by in situ hybridization

In a case of Philadelphia chromosome (Ph1)-negative chronic myeloid leukemia (CML) without the Y chromosome, we investigated the differences, at the molecular level, from Ph1-positive CML. Using Southern blot analysis and in situ hybridization studies, we could demonstrate a rearrangement within the breakpoint cluster region (bcr), and the location of a bcr-abl fusion gene on chromosome 22. To our knowledge, this is the first case of Ph1-negative CML with a loss of the Y chromosome in which the molecular abnormalities are shown to be identical with those in Ph1-positive CML.     

Cytofluorometric detection of chronic myelocytic leukemia supervening in a patient with chronic lymphocytic leukemia

An 82-year-old woman with stage I chronic lymphocytic leukemia presented with systemic symptoms, minimal adenopathy, hepatosplenomegaly, and anemia five years after the initial diagnosis was made and while receiving no therapy. Her white blood cell count was 231,000/mm3 with an absolute neutrophil count of 164,360/mm3 and lymphocyte count of 43,890/mm3. Peripheral blood smear inspection revealed both increased mature lymphocytes and myeloid cells at all stages of maturation. Flow cytometric analysis of forward- and right-angle light scatters demonstrated the presence of two populations of cells, one lymphoid, bearing predominantly lambda light chain surface immunoglobulin and showing phenotypic characteristics of B cell chronic lymphocytic leukemia (HLA-DR-positive, BL-1-positive, BL-2-positive, BL-7-positive, Leu-1-positive, Leu-10-positive, BL-5-negative, BL-6-negative, and OKM1-negative), and another granulocytic population expressing phenotypic features compatible with myeloid lineage (HLA-DR-negative, Leu-1-negative, BL-1-negative, BL-2-negative, BL-7-negative, Leu-10-negative, BL-5-positive, BL-6-negative, OKM1-positive, and surface immunoglobulin-negative). All of the peripheral blood cell metaphases were Philadelphia chromosome-positive after 24 hours of culture, confirming the diagnosis of chronic myelocytic leukemia, whereas all of the Epstein-Barr virus-treated B lymphocyte metaphases showed a normal karyotype after two weeks of culture. In this patient, analysis of surface antigens and immunoglobulin fractions by flow cytometry proved to be useful in recognizing concomitantly expressed leukemic lineages. This approach allows the increasing recognition of the heterogeneity of leukemic populations.     

Fetal erythropoiesis in juvenile chronic myelocytic leukemia

Red cell enzymes, 2,3-diphosphoglycerate (2,3-DPG) and adenosine triphosphate (ATP), were evaluated in a 23-mo-old boy with juvenile chronic myelocytic leukemia (JCML) at the onset of his illness and 6 mo later during the accelerated phase. The activities of the age-dependent red cell enzymes, hexokinase, aldolase, pyruvate kinase, and glucose-6- phosphate dehydrogenase, were elevated, as were the concentrations of red cell 2,3-DPG and ATP, consistent with a young red cell population metabolizing at an increased glycolytic rate. The activities of the non- age-dependent enzymes, glyceraldehyde-3-phosphate dehydrogenase (G3PD), phosphoglycerate kinase, and enolase, were also increased to levels similar to or greater than those observed in term infants. As the illness progressed, the activity of red cell G3PD increased further, and phosphoglucose isomerase activity increased markedly. These results are consistent with the prior suggestion that JCML represents a reversion to "fetal" erythropoiesis.     

染色体核型及血清碱性磷酸酶水平在慢性粒细胞白血病病情预测中作用

目的 研究慢性粒细胞白血病(CML)患者骨髓单个核细胞染色体核型与血清碱性磷酸酶(ALP)水平间变化关系,探讨细胞遗传学变化及ALP水平在患者病情及预后判断中的价值.方法 选择不同时期CML患者,检测其血清ALP,并取骨髓有核细胞采用直接法或24小时培养法、R带显色技术作染色体分析.结果 CML患者ALP水平高于正常对照组,加速期急变期患者复杂核型出现率及血清ALP水平显著高于慢性期患者,且ALP水平与髓内幼稚细胞数水平呈正相关,PH1阳性核型CML患者与复杂核型CML患者血清ALP水平差别不明显.结论 CML患者血清ALP水平及染色体复杂核型反应该期患者疾病特征,对CML疾病分期、预后判断有价值,两者间的一致性有待进一步研究.     

Late-appearing Philadelphia chromosome in two patients with chronic myelogenous leukemia

We describe two patients with typical myelogenous leukemia, who at the beginning of the disease lacked the Philadelphia chromosome in bone marrow cells, and 90 and 42 days later, respectively, its presence was shown in all cells analyzed from that tissue. These findings are compatible with the possibility that at least occasionally Ph1 occurs secondarily in already leukemic cells. The rapid change form Ph1- to Ph1+ CML in one of the patients (42 days), suggests the possibility that in addition to Ph1+ cells enjoying marked selective advantage, this change is induced simultaneously in multiple bone marrow cells.     

慢性粒细胞白血病患者细胞染色体表型与血清LDH、HBDH变化的临床意义

目的 探讨慢性粒细胞白血病(CML)患者细胞染色体表型与血清乳酸脱氢酶(LDH)、γ-羟丁酸脱氢酶(HBDH)变化的临床意义.方法 用生化法检测不同病期CML患者的血清LDH、HBDH,并取其骨髓有核细胞用R带分析法作染色体分析.结果 CML加速期或急变期组复杂核型染色体出现频率及血清LDH、HBDH显著高于慢性期组,两组不同染色体表型患者间的LDH、HBDH无统计学差异.慢性期组复杂核型染色体患者的骨髓原幼细胞高于单纯Ph1 者.LDH、HBDH在加速期或急变期组与骨髓幼稚细胞均呈正相关,在慢性期组与外周血WBC呈正相关.结论 CML患者的血清LDH、HBDH水平及复杂核型染色体出现频率与病情相关,血清酶、细胞核型联合检测有助于更准确地判断CML患者的病情及预后.