Unusual bone scintigraphy in chronic myelogenous leukemia — report of a case showing extensive uptake defect

An extensive ^99mTc-methylene diphosphonate uptake defect was observed on bone scintigraphy in a 35-year-old male with chronic myelogenous leukemia. This type of bone scintigraphy pattern is quite unusual in leukemic patients and we speculate that acute disturbance of blood supply to the bone marrow was probably the cause.     

Avascular necrosis of the femoral head as a complication of chronic myelogenous leukaemia

 A 17-year-old male patient complaining of intense pain in his right hip was found to be suffering from chronic myelogenous leukaemia. Preliminary X-rays and bone scintigraphy did not suggest avascular necrosis of the femoral head. Magnetic resonance imaging (MRI) did, however, reveal leukaemic infiltration of the femoral neck and generalised ischeamia in the femoral head. Further, MRI carried out 4 months later disclosed typical signs of osteonecrosis, despite previous indications of an improvement under chemotherapy. Flattening of the head of the femur appeared in radiographs taken in the 9th month. In the 12th month, recurrence of pain made it necessary to perform a total hip arthroplasty. Anatomo-pathological investigation confirmed both the necrosis and the leukaemic invasion.     

骨髓形态学评价在进展期慢性髓细胞白血病格列卫治疗初期的意义

目的 分析进展期慢性髓细胞白血病(CML)格列卫治疗初期骨髓细胞形态学的变化,探讨其在评价病情变化及指导治疗中的意义.方法 Ph染色体阳性CML进展期患者16例,其中加速期9例、急变期7例,口服格列卫0.4或0.6g/d,分别于治疗前及治疗3、6、9周采集骨髓涂片,常规糖原(PAS)染色,并分析骨髓细胞形态学变化.结果 治疗过程中,多数患者骨髓细胞增生度降低较明显,粒系及原始细胞比例下降,但红系比例增高明显,巨核细胞3周时下降明显;少数患者除了红系仍呈明显增高趋势外,其余上述特征变化不明显,按照诊断标准评价骨髓,可使约1/3患者病情评价出现误差.对外周血白细胞及血小板下降显著的患者,若骨髓粒系、巨核细胞显著降低,则暂时减量或停止格列卫治疗.结论 进展期CML应用格列卫治疗初期骨髓形态学具有特征性的变化,可用于评价病情变化及指导治疗.     

HLA基因多态性与慢性粒细胞白血病的关联性研究

目的探讨人类白细胞抗原（HLA）基因多态性与慢性粒细胞白血病（CML）的关联性。方法采用聚合酶链反应序列特异性引物（PCR-SSP）分型技术对234例CML患者以及1370例无血缘关系健康对照者进行HLA-A/B/C/DRB1/DQB1基因分型,采用病例对照研究方法进行基因频率、单倍型频率以及疾病发生相对危险度（RR）的研究。结果 CML患者中HLA-Cw3及Cw7的基因频率较对照组显著升高（P〈0.05）,其RR分别为1.4777和3.0595。而HLA-Cw4基因型频率则显著降低（P=0.0020）,其RR为0.4331。扩展单倍型A2-B51-Cw14-DR9-DQ9与A11-B13-Cw3-DR12-DQ07的频率亦有显著性差异（P〈0.05）,其RR分别为3.1027和3.1606。两位点单倍型A2-Cw3、A24-Cw3、B15-Cw1与B40-Cw3的频率也存在显著性差异（P〈0.05）,其RR分别为2.5574、2.2544、3.8587及1.6853。结论通过大样本量的病例对照研究HLA基因多态性与CML之间的关联性探讨取得了初步结果：HLA-Cw3及Cw7与CML的易感性相关;HLA-Cw4则与CML发生呈负相关,可能是CML的保护性基因;某些HLA单倍型或扩展单倍型亦与CML的发生有关。     

Avascular necrosis of bone in severe acute respiratory syndrome

AIM: To report the incidence of avascular osteonecrosis (AVN) in severe acute respiratory syndrome (SARS) patients. MATERIALS AND METHODS: Sixty-seven SARS patients who had large joint pain between March 2003 and May 2003 underwent both plain radiographs and magnetic resonance imaging (MRI) examination on the same day. All patients received steroids and ribavirin treatment. All plain radiographs and MR images were analysed by two experienced musculoskeletal radiologists. Any abnormalities, location, extent, morphology, the number, size and signal intensity of lesions were evaluated. RESULTS: Twenty-eight patients were identified with AVN, The mean time to diagnosis of AVN was 119 days after the onset of SARS, or 116 days after steroid use. Three patients had early bilateral AVN of the femoral head, four patients of one femoral head, five patients of the bilateral hips and knees, four patients of the ipsilateral hip and knees, 10 patients of the knee(s), one patient of the right proximal fibula, and one patient of the knees and talus. Results of hip, knee and ankle plain radiographs were negative. CONCLUSION: AVN can occur in the patients with SARS. AVN had a strong association with steroid use. More studies are required to confirm whether the virus itself can also lead to AVN.     

25例AML患者白血病细胞端粒酶活性分析

目的探讨25例急性髓性白血病患者端粒酶活性的变化及意义.方法采用TRAP-ELISA-PAGE法测定端粒酶活性.结果AML患者端粒酶活性(吸光度A值为2.298±1.059)较正常对照(吸光度A值为0.387±0.598)显著增高.结论AML细胞端粒酶的活化可能在白血病的发生中起着重要的作用.     

FANCD2在急性髓系白血病中表达

目的通过检测急性髓系白血病(AML)中FANCD2蛋白的表达,探讨AML的发病机制及其与预后的关系。方法选取2007年9月至2015年7月中国医科大学附属第一医院、第四医院血液科收治的AML患者108例,应用免疫组织化学染色方法检测FANCD2蛋白的表达。结果本组108例AML患者骨髓标本中,FANCD2蛋白在AML初诊组、完全缓解组、复发组及对照组中的阳性表达率分别为6.3%、63.0%、5.0%和71.4%;初诊组与对照组、完全缓解组比较,差异均有统计学意义(P<0.05);复发组与对照组、完全缓解组比较,差异也均有统计学意义(P<0.05);而完全缓解组与对照组比较,差异无统计学意义(P>0.05);初诊组与复发组比较,差异也无统计学意义(P>0.05)。结论 FANCD2蛋白表达的阳性率可作为AML诊断或预后评估的检测指标,与AML的发生、复发相关。     

Avascular necrosis of bone in human immunodeficiency virus infected patients

The objective of this article was to delineate the causes of avascular necrosis (AVN) in patients with human immunodeficiency virus (HIV). HIV-infected patients with pain in large joints were prospectively screened. Patients had radiographs and magnetic resonance imaging of their affected joints. Serum lipids, anticardiolipin antibody levels (IgG, IgM), and hemoglobin electrophoresis were performed on all patients who had radiographic studies. Medical records were screened for factors known to predispose for AVN. Eight patients completed the protocol, and five patients had AVN in seven joints. No common laboratory abnormality was identified in the patients with AVN. All of the patients with AVN had a history of steroid use; four of five patients having taken steroids for HIV-related diseases. The cause of AVN does not appear to be directly related to the disease, but to steroid treatment for manifestations of the disease.     

慢性髓细胞白血病相关蛋白CMLAP的基因克隆化研究

目的了解慢性髓细胞白血病(CML)基因表达谱的规律,并对在CML中特异性高表达的一个新基因进行克隆、分析与鉴定。方法应用基因表达谱芯片技术,比较CML患者和正常人外周血单个核细胞(PBMC)基因的表达差异。检索核苷酸序列数据库(GenBank)和蛋白质一级结构序列数据库(SwissProt),对差异表达的基因进行生物信息学分析,与已知功能基因序列进行同源性比较。根据基因起始密码子的Kozak规则和终止密码子下游保守的多聚腺苷酸信号序列,确定新基因序列,据此设计并合成该基因序列的特异性引物,提取CMLPBMC的总RNA,以RT-PCR技术扩增获得该新基因的全长序列,并对克隆的基因及其编码产物的序列进行分析。结果在CML患者PBMC中克隆一个新的基因,经测序证实,其编码序列全长为1872个核苷酸(nt),编码产物由624个氨基酸残基(aa)组成,命名为CMLAP。在GenBank中注册,注册号为AY762229。结论基因表达谱芯片技术与生物信息学技术相结合,发现并鉴定、克隆了在CML中高表达的新基因CMLAP,为进一步研究CML发生发展的分子生物学机制奠定基础。     

The skeletal manifestations of chronic myelogenous leukemia

Skeletal lesions were radiographically apparent in 6 of 36 (16%) patients with chronic myelogenous leukemia. The spectrum of radiographic changes including diffuse osteoporosis, focal osteolytic and osteoblastic lesions, chloroma, and arthritis, and their clinical behavior is discussed.     

Avascular necrosis of the epiphysis of the first metatarsal bone

We report a case of avascular necrosis of the epiphysis of the right first metatarsal in a 6-year-old boy. Radiographs showed sclerosis, collapse and a crescent sign in the epiphysis. The diagnosis was confirmed by magnetic resonance imaging and scintigraphy. Arch support was the therapy of choice. Six months after the onset of symptoms, a definite reossification was present. To our knowledge, this is the first radiological report of avascular necrosis of the epiphysis of the first metatarsal bone in the world literature, which prompted a review of the osteochondroses and their etiology.     

格列卫治疗慢性粒细胞白血病急变期患者的临床疗效及毒性反应观察

目的评价格列卫治疗慢性粒细胞白血病急变期患者的疗效及毒性反应。方法36例患者中31例患者单用格列卫治疗,有5例加用亚砷酸治疗,根据患者耐受情况调整格列卫剂量。开始每2天复查外周血涂片、血常规,每周复查肝肾功能,1个月后每周复查外周血涂片、血常规,每2周复查肝肾功能,每1个月进行骨髓细胞形态学检查,每3个月进行细胞遗传学检查,记录治疗过程中的不良反应。结果1个月时血液学缓解率（包括CHRT和NEL）66.7%,服用格列卫3个月后进行细胞遗传学检查,5例附加染色体异常消失,其中部分细胞遗传学缓解3例,完全细胞遗传学缓解2例。血液学不良反应以白细胞、血小板减少多见,非血液学不良反应以恶心、呕吐、水肿多见,其次为骨骼肌酸痛、乏力、头昏常见,而胸腔积液、头痛、皮疹等少见。结论尽管格列卫是一种较安全的药物,其治疗慢粒急变期患者近期疗效明显,但远期疗效并不理想。     

Tophaceous gout in an amputation stump in a patient with chronic myelogenous leukemia

Gout is a common rheumatologic disorder that can have an unusual clinical presentation. This case report describes the development of a gouty tophus at a site of remote traumatic forearm amputation in a patient with chronic myelogenous leukemia (CML). It further addresses the imaging characteristics of tophaceous gout as well as the differential diagnostic considerations as regards both the imaging findings and the clinical presentation.     

Unusual bone scintigraphy in chronic myelogenous leukemia--report of a case showing extensive uptake defect

An extensive 99mTc-methylene diphosphonate uptake defect was observed on bone scintigraphy in a 35-year-old male with chronic myelogenous leukemia. This type of bone scintigraphy pattern is quite unusual in leukemic patients and we speculate that acute disturbance of blood supply to the bone marrow was probably the cause.