氟马替尼与伊马替尼治疗初发慢性髓性白血病慢性期患者的有效性及严重血液学不良反应发生率比较

目的比较氟马替尼与伊马替尼治疗初发慢性髓性白血病(CML)慢性期患者的治疗反应、结局以及严重血液学不良反应发生率。方法回顾性收集来自国内76个中心自2006年1月至2022年11月期间确诊、年龄≥18岁、确诊后6个月内接受氟马替尼或伊马替尼作为一线治疗且临床资料相对完整的CML慢性期病例。通过倾向性评分匹配(PSM)减少一线酪氨酸激酶抑制剂(TKI)药物选择偏倚, 比较两种TKI的治疗反应及结局。结果研究最终纳入4 833例接受伊马替尼(4 380例)和氟马替尼(453例)作为一线治疗的成人CML慢性期患者。伊马替尼治疗组中位随访54(IQR:31～85)个月, 7年完全细胞遗传学反应(CCyR)、主要分子学反应(MMR)、分子学反应4(MR4)和分子学反应4.5(MR4.5)累积获得率分别为95.2%、88.4%、78.3%和63.0%。7年无治疗失败生存(FFS)率、无进展生存(PFS)率和总生存(OS)率分别为71.8%、93.0%和96.9%。氟马替尼治疗组中位随访18(IQR:13～25)个月, 2年CCyR、MMR、MR4和MR4.5累积获得率分别为95.4%、86.5%、...     

M蛋白阳性患者314例体液免疫特征分析

[目的]分析314例血清蛋白电泳M蛋白阳性患者血尿免疫学特征及其相关性。[方法]对华西医院门诊及住院患者采用琼脂糖凝胶电泳法作血清蛋白电泳检测,检出M蛋白带的患者,再作免疫球蛋白G、A、M、血轻链Kappa(κ)、Lambda(λ)和Kappa/Lambda(κ/λ)比值、尿游离轻链Kappa(κ)、Lambda(λ)检测,用免疫固定电泳鉴定M蛋白类型,采用t检验、配对秩和检验和相关分析比较其血尿蛋白免疫学特征及其相关性。[结果]3156例血清蛋白电泳筛检出M蛋白阳性314例,其中IgG型所占比例最大(66.6%)。血清蛋白电泳后,IgG和IgM型M带主要分布于γ区,较少分布在α2和β区;IgA型M带主要分布在γ区,其次分布在β区,较少分布在α2区。IgG、IgA、IgM型患者对应Ig的血清浓度均显著升高(P﹤0.01),IgG和IgA型患者的非对应Ig血清浓度明显低于正常(P﹤0.01),IgM型患者的IgG浓度明显低于正常(P﹤0.01),而IgA浓度略有降低但并不显著。M蛋白类型对应轻链的血清浓度均显著增高(P﹤0.01),非对应轻链均在正常范围内;各类型患者血清κ/λ值均明显异常。以免疫固定电泳的结果作为参考,观察各指标检测M蛋白的能力,其敏感性依次为血清κ/λ98.1%,血清Ig97.1%,血清轻链96.2%,尿轻链78.1%。[结论]应用免疫固定电泳结合血清蛋白电泳及免疫球蛋白定量等方法可以比较敏感地检测出M蛋白,并对其进行分类、分型,为临床诊断和治疗提供重要依据。     

大颗粒淋巴细胞增多症16例临床及免疫表型分析

大颗粒淋巴细胞增多症(Lymphoproliferative disease ofgranular lymphocytes,LDGL)是一种比较少见的淋巴细胞增殖性疾病,占T/NK细胞性恶性疾病的2%～5%[1].其特征为外周血大颗粒淋巴细胞(LGL)持续性的升高.该病由Loughran于1985年首次报道[2],迄今为止仅报道400余例.     

急性肿瘤溶解综合征1例并文献复习

急性肿瘤溶解综合征(Acute tumor lysis syndrome, ATLS)是肿瘤治疗过程中出现的一种具有潜在致命风险的急性并发症,是由于肿瘤细胞大量溶解,快速释放细胞内物质,超过肝脏代谢和肾脏排泄的能力,导致代谢异常和电解质紊乱,引起以高尿酸血症、高钾血症、高磷血症和肾功能不全为主要表现的症候群[1,2].ATLS可以发生于各种肿瘤治疗后,病情进展迅速,死亡率高.本文报道严重急性肿瘤溶解综合征1例并对文献进行复习,以提高对ATLS的诊断、治疗水平.     

淋巴结细针穿刺标本的流式细胞术分析

Objective Comparative evaluation of flow cytometric immunophenotyping in the diagnosis and differentiation of lymphadenopathy,lymphoma and reactive lymphoid hyperplasia. Methods Ninty-nine fine-needle aspiration specimens from patients with tentative clinical lymphoprofierative disorders were consecutively analyzed by both cytology and flow cytometry with histology results as the gold standard. The three color antibodies including CD3,CD3,CD4,CD5,CD10,CD19,CD20,CD23,CD45,K,λ,FMC7 and CD34 were used for cell composition evaluation and cells with abnormal phenotype. Lymphoma cases were classified according to new WHO classification and subtypes were categorized by immunophenotypic analysis. The results from flow cytometry and cytology were compared. Results By cytological study, 40 of 99 cases were diagnosed with lymphoma, 29 cases were diagnosed with metastatic carcinoma, and 30 cases were diagnosed with reactive lymphoid hyperplasia, necrosis or tuberculosis. Among them, 2 non-Hodgkin lymphoma(NHL) cases were misdiagnosed as reactive lymphoid hyperplasia by cytology. Biopsy was performed in 18 cases of NHL including 16 B-NHL and 2 T-NHL By flow cytometry study, 35 of 99 eases were diagnosed with lymphoma, including 4 cases of lymphoblast lymphoma, 1 case of T-cell lymphoma, and 30 eases of other B-NHL For those 30 cases of B-NHL, 28 cases showed monoclonal light chain expression, and k: λ orλ: k atios exceed 3: 1, and B-cell proportion was (73. 2±27. 2)%. Twenty-six cases could be sub-classified by immunophenotyped. Among 16 histologically confirmed B-NHL cases, only 2 cases diagnosed with follicular lymphoma showed discrepancy with flow cytometry results. In all cases diagnosed with reactive lymphoid hyperplasia and metastasis carcinoma , no abnormal lymphocytes can be found, and k: λ or k: λ ratios were less than 3: 1. Conclusions Fine-needle aspiration analysis with flow eytometrie immunophenotyping can be helpful in diagnosis and differential diagnosis as well as sub-classification of NHL     

成人原发性中枢神经系统淋巴瘤和原发性眼内淋巴瘤诊断及治疗指南

制定该指南的成员由英国医学专家和患者代表推选.以中枢神经系统和眼内淋巴瘤为关键词在medline和EMBASE数据库系统地检索了1950年～2007年4月期间以英文发表的文章.该指南由编写小组起草,随后由英国血液学标准委员会(BCSH)血液一肿瘤组的成员修订达成一致意见.     

万珂为主化疗方案治疗多发性骨髓瘤的临床研究

目的 研究蛋白酶体抑制剂硼替佐米(万珂,Velcade,V)为主的化疗方案治疗多发性骨髓瘤(MM)的疗效和不良反应. 方法 MM患者接受万珂为主的联合方案进行化疗.疗效评定按照EBMT/ABMT标准.结果 可评估疗效者12例,初治8例,复发难治4例.治疗总体反应率100%(12/12),其中,完全缓解(CR)5例(41.7%),接近完全缓解(nCR)2例(16.7%),部分缓解(PR)5例(41.7%).初治患者达到最佳反应所需疗程中位数为2个(2～3个),复发难治患者达到最佳反应所需疗程中位数为4个(3～5个).中位随访11月(4～27月),所有患者均存活.不良反应多为1～2级,包括周围神经病变(41.7%)、血小板减少(33.3%)、恶心呕吐(33.3%)及呼吸道感染(16.7%),经对症处理后好转.结论 万珂为主的化疗方案治疗初治和复发难治MM病例,起效较快,反应率和完全缓解率较高,不良反应轻,患者耐受好,值得推广.     

淋巴结细针穿刺标本的流式细胞术分析

Objective Comparative evaluation of flow cytometric immunophenotyping in the diagnosis and differentiation of lymphadenopathy,lymphoma and reactive lymphoid hyperplasia. Methods Ninty-nine fine-needle aspiration specimens from patients with tentative clinical lymphoprofierative disorders were consecutively analyzed by both cytology and flow cytometry with histology results as the gold standard. The three color antibodies including CD3,CD3,CD4,CD5,CD10,CD19,CD20,CD23,CD45,K,λ,FMC7 and CD34 were used for cell composition evaluation and cells with abnormal phenotype. Lymphoma cases were classified according to new WHO classification and subtypes were categorized by immunophenotypic analysis. The results from flow cytometry and cytology were compared. Results By cytological study, 40 of 99 cases were diagnosed with lymphoma, 29 cases were diagnosed with metastatic carcinoma, and 30 cases were diagnosed with reactive lymphoid hyperplasia, necrosis or tuberculosis. Among them, 2 non-Hodgkin lymphoma(NHL) cases were misdiagnosed as reactive lymphoid hyperplasia by cytology. Biopsy was performed in 18 cases of NHL including 16 B-NHL and 2 T-NHL By flow cytometry study, 35 of 99 eases were diagnosed with lymphoma, including 4 cases of lymphoblast lymphoma, 1 case of T-cell lymphoma, and 30 eases of other B-NHL For those 30 cases of B-NHL, 28 cases showed monoclonal light chain expression, and k: λ orλ: k atios exceed 3: 1, and B-cell proportion was (73. 2±27. 2)%. Twenty-six cases could be sub-classified by immunophenotyped. Among 16 histologically confirmed B-NHL cases, only 2 cases diagnosed with follicular lymphoma showed discrepancy with flow cytometry results. In all cases diagnosed with reactive lymphoid hyperplasia and metastasis carcinoma , no abnormal lymphocytes can be found, and k: λ or k: λ ratios were less than 3: 1. Conclusions Fine-needle aspiration analysis with flow eytometrie immunophenotyping can be helpful in diagnosis and differential diagnosis as well as sub-classification of NHL     

组织胞浆菌病一例

患者女，32岁，小学教师。因乏力伴间歇性发热、腹胀2个月，加重1个月于2002年5月入院。居住四川，无外出史。休检：休温39℃，脉搏124次/min，血压98/50mmHg(1mmHg=0.133kPa)。中度贫血貌，四肢散在出血点及陈旧性淤斑，浅表淋巴结无肿大。心肺未见异常。肝肋下6cm,剑下7cm。脾肋下Ⅰ线9cm,Ⅱ线7cm,Ⅲ线-1.5