健康教育在白血病护理中的应用效果

目的评价血液科白血病疾病护理中应用健康教育的临床效果,为白血病护理工作提供参考。方法选择医院收治的白血病患者,总计60例。进行白血病患者随机分组护理,对照组与试验组各30例白血病患者,分别采取常规护理和常规护理+健康教育,比较2组白血病患者的负性情绪、自我效能状况评分以及护理满意度、护理依从率。结果试验组白血病患者干预后SAS、SDS、GSES评分均明显优于对照组,护理满意度与护理依从率均明显高于对照组,指标差异具有统计学意义,P>0.05。结论血液科白血病治疗期间配合落实常规护理、健康教育可以在提高患者自我效能的同时提高患者的依从性,符合患者身心护理需求,整体护理效果显著。     

Absence of FGFR3–TACC3 rearrangement in hematological malignancies with numerical chromosomal alteration

FGFR–TACC, found in different tumor types, is characterized by the fusion of a member of fibroblast grown factor receptor (FGFR) tyrosine kinase (TK) family to a member of the transforming acidic coiled-coil (TACC) proteins. Because chromosome numerical alterations, hallmarks of FGFR–TACC fusions are present in many hematological disorders and there are no data on the prevalence, we studied a series of patients with acute myeloid leukemia and myelodysplastic syndrome who presented numerical alterations using cytogenetic traditional analysis. None of the analyzed samples showed FGFR3–TACC3 gene fusion, so screening for this mutation at diagnosis is not recommended.     

Is dose modification or discontinuation of nilotinib necessary in nilotinib-induced hyperbilirubinemia?

Nilotinib is a specific breakpoint cluster region-Abelson leukemia virus-tyrosine kinase inhibitor that is used as an effective first- or second-line treatment in imatinib-resistant chronic myelogenous leukemia (CML) patients. Hepatotoxicity due to nilotinib is a commonly reported side effect; however, abnormal liver function test (LFT) results have been reported in asymptomatic cases. When alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels are more than five-fold the upper limit of the normal (ULN) or when the serum total bilirubin level is more than three-fold the ULN, dose modification or discontinuation of nilotinib is recommended, resulting in decreased levels of hematological indicators in certain patients with CML. Nilotinib-induced hyperbilirubinemia typically manifests as indirect bilirubinemia without elevated ALT or AST levels. Such abnormal liver functioning is thus not attributed to the presence of a true histologic lesion of the liver. The underlying mechanism may be related to the inhibition of uridine diphosphate glucuronosyltransferase activity. Therefore, nilotinib dose adjustment is not recommended for this type of hyperbilirubinemia, and in the absence of elevated liver enzyme levels or presence of abnormal LFT findings, physicians should consider maintaining nilotinib dose intensity without modifications.     

A new era of immuno-oncology in acute myeloid leukemia - antibody-based therapies and immune checkpoint inhibition

Acute myeloid leukemia (AML) remains a therapeutically challenging malignancy with high rate of relapse and poor outcomes. There has been increased understanding of the molecular characteristics of AML and the various roles of the immune system in its pathogenesis, the result of which has led to the study and development of multiple immune-based approaches for this disease. In this review, we aim to provide an overview of the recent advancements made in antibody-based approaches to the treatment of AML including monoclonal antibodies, antibody-drug conjugates, and immune checkpoint inhibition. In addition, we provide insight and discuss the promise of these agents, some of which may soon enter the therapeutic armamentarium we currently employ against this lethal disease.     

WT1基因与急性髓系白血病的预后及主动特异性免疫治疗的研究进展

WT1基因位于人类染色体11p13，在80%的急性髓系白血病（acute myeloid leukemia，AML）患者中高表达，是AML预后不良的分子标志，可作为AML预后评估和微小残留病变（minimal residual disease，MRD）监测的有效指标。由于WT1在AML中均有异常高表达，故认为是一种AML抗原，可作为特异性免疫治疗的新靶点。一些小规模的临床试验证实以WT1为靶点的免疫治疗是有效的、安全的，这些免疫治疗可作为那些有高危复发风险及初始标准化疗失败的AML患者的辅助治疗。本文就近几年WT1与AML的预后及有关以WT1为靶点的主动特异性免疫治疗的研究进展予以综述。     

Translocation between chromosome 5q35 and chromosome 11q13 – an unusual cytogenetic finding in a primary refractory acute myeloid leukemia

Cytogenetic abnormalities are observed in approximately two‐thirds of patients with acute myeloid leukemia (AML). Chromosome rearrangements are associated with specific subtypes of AML and associated prognosis. We report a patient with AML, M2, who was primarily refractory to standard induction chemotherapy with idarubicin and cytarabine. Flow cytometry of a bone marrow aspirate showed aberrant expression of B‐cell markers including CD19. Cytogenetic studies disclosed a translocation between 5q35 and 11q13. Fluorescence in situ hybridization analyses demonstrated that neither the NSD1 nor MLL genes were involved in this case. Further study is required to define conclusively the genes involved and their contribution to pathogenesis in this case.     

MPO和NAT2基因多态性与成人急性白血病易感性

目的研究髓过氧化物酶（MPO）和N-乙酰基转移酶2（NAT2）基因多态性与成人急性白血病易感性的关系.方法用1∶1配对病例-对照研究方法,收集成人急性白血病患者和对照各139例,应用聚合酶链-内切酶片段（PCR-RFLP）方法分析病例组和对照组MPO和NAT2的基因多态性,比较不同基因型与成人急性白血病易感性.结果 MPO-463A等位基因分布频率病例组低于对照组,MPO（G-463A）各基因型在病例组与对照组中的分布差异有统计学意义（χ^2=7.026,P〈0.05,OR=0.505,95%CI=0.325～0.847）.NAT2乙酰化表型频率在病例组与对照组的分布差异无统计学意义（χ^2=2.260,P〉0.05）;但NAT2＊5 481T等位基因和NAT2＊6 590A等位基因分布频率病例组高于对照组（P〈0.05）.结论 MPO与成人急性白血病易感性相关,携带MPO（G-463A）突变基因型（GA/AA）个体可降低白血病的发病风险;NAT2乙酰化表型可能与白血病的易感性无关,但NAT2＊5（C481T）、NAT2＊6（G590A）单核苷酸突变频率病例组明显高于对照组.     

定期骨髓象观察指导急性淋巴细胞白血病诱导缓解治疗

目的评价定期骨髓象检查对急淋诱导化疗的指导意义。方法对引例初治成人急淋患音，于诱导化疗首疗程期间定时作骨髓象观察，用这两个时限的骨髓白血病细胞比率及／或白血病细胞减少指数指导化疗。结果本组完全缓解（CR）率82.4%（42/51），其中首疗程CR率60.8%（31／51）。结论诱导化疗期定时骨髓象观察指导化疗有助于提高急淋CR率及首疗程CR率。     

Acute Leukemia in an Infant with Marfan's Syndrome: A Case Report

We describe a familial case of Marfan's syndrome with associated intrathoracic stomach detected during the neonatal period. The patient developed a primitive leukemia at 3 months of age. Acute leukemia in a patient with Marfan's syndrome has not previously been reported.