Hairy cell leukemia

Hairy cell leukemia (HCL) is a chronic B-cell lymphoproliferative disorder characterized by pancytopenia and variable infiltration of the reticuloendothelial system with "hairy" lymphocytes. HCL is more common in men than women and has a median age of diagnosis of 52 yr. Typically, patients with HCL respond well to purine analog-based therapy. The purpose of this review will be to establish the current status of HCL with respect to its pathophysiology, diagnosis, management, and future directions.     

Emerging therapeutic options for Philadelphia-positive acute lymphocytic leukemia

Acute lymphocytic leukemia (ALL) is a heterogeneous group of disorders that are associated with a cure rate of > 80% in children. The prognosis in adults is considerably inferior, with age, disease bulk, leukemia karyotype and immune phenotype being prognostically relevant. Adult ALL treatment programs include induction, intensified consolidation and maintenance phases with CNS prophylaxis. The addition of imatinib in patients with BCR-ABL-positive ALL has improved the prognosis of this subgroup, but their survival is still poor. Initial data on the second-generation BCR-ABL inhibitors, dasatinib and nilotinib, indicate a potentially greater efficacy than imatinib, but the improvement is likely to be modest. The overall efforts in terms of developmental therapeutics in ALL are very modest and not in keeping with the urgent need for improvement. Most agents being investigated have mechanisms of action similar to those of existing agents for ALL therapy and thus represent modest opportunities to improve results. Of such agents, data on BCR-ABL inhibitors, sphingosomal vincristine, pemetrexed, talotrexin, annamycin and ABT-751 are reviewed.     

腹腔镜在小儿离断性肾盂成形术中的应用与随访

目的 探讨腹腔镜下离断性肾盂成形术的效果及适应证.方法 总结21例经腹腔途径行腹腔镜离断性肾盂成形术治疗肾盂输尿管连接部狭窄(UPJO)所致肾积水的经验,对比同期开放手术治疗的35例患儿术后影像学.结果 第一例腹腔镜肾盂成形术历时4 h 50 min,随后的20例为1 h 40 min～2 h 30 min,术中出血量5～10 ml.20例术后3～5 d出院,恢复好.1例术后7 d因患儿外伤性肾盂内出血,血块堵塞双J管,致肾盂漏尿、形成尿腹,遂行开放探查术;术中见吻合口良好,清除肾盂内血块,冲洗双J管,放置.肾造瘘管,7 d后拔除肾造瘘管出院.21例患儿术后4～6周拔除双J管.2组患儿均分别在术后1个月、3个月、6个月行B超或CTU检查.腔镜组吻合口通畅率为95.2%(20/21),开放组为100%(35/35),二者差异无显著性意义.结论 熟练掌握腹腔镜缝合技术,是圆满完成经腹腔镜离断性肾盂成形术的基础.腹腔镜下手术创伤轻微,手术效果与传统的开放手术无差别;在应用腹腔镜肾盂成形术的初期,宜选择中度以下肾盂扩张的病例,待熟练后,逐步应用于重度积水病例,确保手术效果.     

老年性痴呆与血管性痴呆患者睡眠障碍的对照研究

目的:研究老年性痴呆(AD)和血管性痴呆(VD)患者睡眠障碍的特点及相关性。方法:对31例AD患者和30例VD患者采用阿森斯(Athens)失眠量表调查评分及临床资料比较分析。对研究对象进行筛选分组。从8个方面详细记录睡眠情况,按量表现规定进行评定。结果:AD患者和VD患者总体睡眠状况比较有显著性差异(t=2.251,P<0.05)。睡眠状况各因子比较,在总睡眠质量、白天情绪、白天思睡等3个方面无显著性差异(P>0.05)。在早醒、白天身体功能方面,两者有极显著差异(P<0.01)。在入睡时间、夜间苏醒、总睡眠时间等两者存在显著性差异(P<0.05)。结论:AD患者和VD患者都有睡眠障碍,AD患者在早醒、白天身体功能、夜间苏醒、总睡眠时间等方面都比VD患者差,而VD患者在入睡时间上比AD患者明显延迟。     

Evaluation of the detection of PML-RARα fusion gene in acute promyelocytic leukemia to monitor minimal residual disease

Objective To investigate the kinetics of PML-RARα fusion gene in acute promyelocytic leukemia(APL)to monitor minimal residual disease(MRD). Methods In induction therapy,consolidation and maintenance therapy courses, PML-RARα fusion gene was performed by RT-PCR. Results The long-term follow-up of 18 cases achieved complete remission (CR),two cases experienced molecular relapse. One case relapsed at 4 months after CR1 and achieved CR2 after induction therapy. However, molecular and hematology relapsed again at 2 months after CR2 and re-achieved CR3. The other case relapsed at 74 months after CR1 and achieved CR2 after induction treatment, who had survived for 106 months until the end of follow-up. Conclusion RT-PCR assay for detection of PML-RARα should be performed regularly during CR period so as to find molecular relapse eady. Hematological relapse could potentially be averted through treatment modification according to molecular monitoring results of PML-RARα.     

The trouble with family medicine

BACKGROUND: The trouble with family medicine is that the perceptual framework it uses to view the phenomena of health and illness is at variance with the frameworks traditionally used by medicine generally. This creates difficulties in communication between those in family medicine and those in other disciplines, and sometimes leads to misunderstanding of the nature of the discipline of family medicine and its place in the health care system. Those who practise family medicine need to be 'multilingual', able to understand and speak the language and use the metaphors of family medicine, yet equally able to use the language and metaphors of other disciplines. OBJECTIVES: This paper, which begins with a clinical scenario, reviews the contemporary biomedical paradigm, proposes an alternative, and examines the conceptual frameworks which underpin the discipline of family medicine.      

Rapid mobilization of hematopoietic progenitor cells in rhesus monkeys by a single intravenous injection of interleukin-8

Interleukin-8 (IL-8) is a chemoattractant cytokine involved in chemotaxis and activation of neutrophils. Because in vivo administration of IL-8 induces mobilization of hematopoietic stem cells in mice, we assessed the mobilizing properties of IL-8 in rhesus monkeys. Recombinant human IL-8 was administered as a single intravenous injection at doses of 10, 30, and 100 micrograms/kg to rhesus monkeys (age, 2 to 3 years; weight, 2.5 to 4.5 kg). Venous blood samples were obtained at time intervals ranging from 1 to 480 minutes after IL-8 administration. Cell counts, colony-forming unit-Mix assays, and fluorescence-activated cell sorter analysis were performed. Plasma was harvested to assess IL-8 levels. A time-controlled bolus intravenous injection of 100 micrograms IL-8 per kilogram of body weight resulted in peak IL-8 plasma levels up to 5 micrograms/mL. The calculated half-time life of free IL-8 was 9.9 +/- 2.2 minutes. IL-8 injection resulted in instant neutropenia that was due to pulmonary sequestration, as shown using 99mTc-labeled leukocytes. Within 30 minutes after IL-8 injection, neutrophilia developed with counts up to 10-fold greater than baseline levels. The numbers of hematopoietic progenitor cells (HPCs) increased from 45 +/- 48/mL to 1,382 +/- 599/mL of blood at 30 minutes after injection of 100 micrograms IL-8 per kilogram of bodyweight (mean +/- SD, n = 8). Individual animals showed 10- to 100-fold increase in numbers of circulating HPCs that returned to almost pretreatment values (92 +/- 52 CFU/mL) at 240 minutes after the injection of IL-8. Immunophenotyping showed no significant changes in lymphocyte (sub)populations. A second bolus injection of IL-8 with an interval of 72 hours resulted in similar numbers of mobilized stem cells as observed after the first injection, showing that no tachyphylaxis had occurred. We conclude that IL-8 induces mobilization of HPCs from the bone marrow of rhesus monkeys in a rapid and reproducible fashion. Therefore, IL-8 may be a potentially useful cytokine in the setting of blood stem cell transplantation.