Clinical analysis of 78 cases of paroxysmal nocturnal hemoglobinuria diagnosed in the past ten years

Objective To learn more about the clinical and laboratory features of patients with paroxy smal nocturnal hemoglobinuria (PNH) diagnosed in the past ten years. Methods Clinical and laboratory data for 78 cases of PNH diagnosed from January 1990 to November 1999 in our hospital were analyzed retrospectively. Results In comparison with PNH cases reported in the 1980s, the newly diagnosed PNH cas es showed the following features: (1) older age of disease onset (from 27 to 34 years); more female cases (from 18. 5% to 38. 5%); more cases without hemoglobin uria (from 24. 2% to 38. 5%). (2) No positive family hereditary history. (3) Bone marrow dysplasia, abnormal karyotype and negative sister chromatid differen tiation were found in 19. 2%, 12. 2% and 8. 9% of the PNH patients, respectively . 12. 3% of the patients had bone marrow hypoplasia, and most of them had no he moglobinuria. Ham’s tests were negative in about 34. 2% of the cases. CD55 and CD59 on peripheral blood cells were deficient in 100. 0% of the cases, suggesti ng that CD55 and CD59 tests can improve the diagnosis of PNH. (4) Adrenocortic al hormone was effective in 83. 8% of the patients, 54. 2% of whom relapsed with in one year. Eight refractory and relapsed patients were treated with low dose chemotherapy (MP therapy: Melphalan 2-6 mg·d; Prednisone 0. 5 mg·kg(-1) ·d(-1) ). Five (62. 5%) of them showed positive responses. Bone marrow failure and other side effects were not serious in this group of patients Conclusions PNH, an acquired blood disease seen more often among adult males, can be diagno sed more sensitively by hemocyte member CD55 and CD59 tests and treated more eff ectively with adrenocortical hormone or low dose chemotherapy.     

骨髓增生异常综合征患者T淋巴细胞功能研究进展

骨髓增生异常综合征 (MDS)是一组恶性克隆性造血干 祖细胞疾病 ,可以有一系或多系造血异常 ,极易发展为急性白血病。有研究表明MDS患者的免疫系统也有异常改变 ,主要表现为细胞免疫异常 :T细胞数目异常、CD4 与CD8 亚群比例异常、细胞毒性T淋巴细胞 (CTL)数量和杀伤肿瘤细胞功能减低、Th细胞中Th1与Th2细胞比例失衡、T细胞分泌功能 (如IL 2、TNF α)异常。免疫异常与疾病的恶性程度有明显关系。现对该方面的研究进展综述如下。1　T淋巴细胞成熟障碍　MDS恶性造血不仅累及髓系 ,在许多病例中也累及淋巴细胞系统。Tsukamoto等[1…     

反应性噬血细胞综合征 美国迈阿密大学医学院血液病中心病例讨论纪实

(1989年10月20日中午,美国迈阿密大学医学院血液病中心会议室) Dr.A:今天讨论急诊病例的诊断及治则。病史:男性,58岁.白人,已婚,发热、乏力、纳差一周。查体:体温39℃:面色苍黄,四肢散在出血点,两腋及腹股沟淋     

免疫相关性全血细胞减少症患者骨髓B淋巴细胞数量、功能及凋亡

目的检测免疫相关性全血细胞减少症(IRP)患者骨髓B淋巴细胞的数量、功能及凋亡相关蛋白水平,以探讨B淋巴细胞在IRP发病中的作用。方法采用流式细胞术测定25例初诊患者,15例治疗后血象恢复正常的患者和10例正常人骨髓总B淋巴细胞、CD5~+B淋巴细胞细胞的数量,B淋巴细胞胞浆内免疫球蛋白IgM的表达以及B淋巴细胞膜表面凋亡相关蛋白Fas抗原(CD95)、胞浆内抗凋亡蛋白bcl—2的表达。结果 IRP初诊患者总B淋巴细胞、CD5~+B淋巴细胞比例显著高于治疗后血象恢复组和正常人(P均<0.05),后两组相比无显著性差异(P>0.05);初诊患者骨髓B淋巴细胞免疫球蛋白IgM的表达也明显高于其他两组。三组患者B淋巴细胞Fas抗原的表达率无明显区别(P均>0.05);初诊患者B淋巴细胞胞浆内bcl—2的表达率显著高于其它两组(P均<0.05),治疗后血象恢复组也显著高于正常对照组(P<0.01);各组间B淋巴细胞凋亡相关指数(CD19~+CD95~+/CD19~+Bcl-2~+)比较,初诊组显著低于治疗后血象恢复组和对照组(P均<0.01),治疗后血象恢复组也显著低于对照组(P<0.05);B淋巴细胞的比例与治疗起效时间呈正相关(r=0.69,P<0.01)。结论 IRP患者自身抗体的产生可能与B淋巴细胞及其亚群数量异常、功能亢进及凋亡受抑有关。     

对造血系统肿瘤性疾病MDS认识的新飞跃——简评WHO关于MDS分类标准

新世纪伊始,世界卫生组织(WHO)正式颁布了系列丛书《WHO肿瘤分类》,其中《造血和淋巴系统肿瘤分类》简明扼要地规范了近百年来人们对恶性血液病("血癌")的不同命名、分类及认识,特别是关于骨髓增生异常综合征(MDS)--这个刚命名20余年的血液病的新分类标准,引起了界内学者的极大关注。     

阵发性睡眠性血红蛋白尿症的治疗

阵发性睡眠性血红蛋白尿症 (ＰＮＨ)是由体细胞Ｘ染色体连锁、ＰＩＧ Ａ基因突变导致的一种获得性血液学紊乱 ,以一个或多个葡萄糖磷脂酰肌醇 (ＧＰＩ)锚定蛋白 (ＧＰＩ ＡＰｓ)缺失或减少的造血细胞克隆的扩增为特征 ,临床主要表现为溶血、血栓形成和造血功能障碍。ＰＮＨ的常规治疗手段有 :肾上腺皮质激素、雄激素、红细胞输入、免疫抑制剂以及抗凝治疗。肾上腺皮质激素至今仍是治疗ＰＮＨ的一种主要药物 ,但对激素无效或依赖的难治性或复发性ＰＮＨ如何治疗 ,一直是棘手的难题。现就有关ＰＮＨ治疗及并发症防治等方面研究进展综述如下。1…     

骨髓单个核细胞Coombs试验阳性的血细胞减少患者补体水平变化及其意义

Objective To investigate the variation of bone marrow complement level in cytopenia pa-tients with positive BMMNC-Coombs test(CBCPC), and probe the role of complement in destroying hemato-poietic cells of CBCPC patients. Methods One hundred and twenty-four patients with CBCPC and twenty-three healthy donors as controls were enrolled in this study. The levels of CI-150, C3, C4, C5b-9 were tested with ELISA. The auto-antibodies on bone marrow hematupoietic cells (BMHC) were examined with flow cy-tometry. Results The level of C5b-9 in bone marrow(BM) of untreated CBCPC patients [(119.8 ± 54.0)μg,/L] was significantly higher than that of recovered patients [(100.7 ± 33.4) μg/L] or normal controls [(93.9 ± 28.8) μg/I.] (P < 0.05). The levels of CH50 in BM of untreated or recovered CBCPC patients [(33.3 ± 11.5) kU/L, (30.8 ± 10.3) kU/L] were significantly higher than that of normal controls [(24.1 ±6.4) kU/L] (P < 0.05). The level of C3 in BM of untreated or recovered CBCPC patients [(4.9 ± 2.2) mg/ L], (5.0 ± 3.5) mg/L] was significantly lower than that of normal controls [(7.0 ± 5.6) mg/L] (P < 0.05). The level of complement in peripheral blood was consistent with that in BM. CH50 in BM of CBCPC patients was negatively correlated with their C3 (r = - 0. 303, P = 0. 007) and positively correlated with their C5b-9(r = 0. 241, P = 0. 003) levels. The level of C5h-9 in BM of CBCPC patients was higher in the BMHC-IgM positive group [(117.6 ± 55.7) μg/L] than in the BMHC- IgM negative group [(99.2 ± 26.2)μg/L] (P < 0. 05). The positive rate of CD34+ -IgG or CD34 + -IgM of CBCPC patients was positively corre-lated with their C5 b-9 level (r = 0. 593, P = 0.000, r = 0. 326, P = 0. 049). The reticulocyte percentage (r =0. 421, P = 0.000) and serum indirect bilirubin level (r = 0. 230, P = 0. 032) of CBCPC patients were posi-tively correlated with their CHSO level. Conclusions The hematocytopenia of CBCPC patients might be re-lated to the hematopoietic cells destruction caused by auto-antibedy activated complements.     

阵发性睡眠性血红蛋白尿症实验室诊断

阵发性睡眠性血红蛋白尿症(PNH)是一种后天获得 的血细胞膜缺陷的克隆性疾病,临床上可表现血管内溶 血、栓塞、全血细胞减少等,有时可出现骨髓衰竭[1]。现 已证实,大多数PNH患者异常克隆与正常造血并存,使本 病的诊断和发病机制的研究受到限制。PNH患者红细胞 膜表面缺乏加速衰变因子(DAF,CD55)、反应性溶血的膜 抑制物(MIRL,CD59)和C8结合蛋白(或称同种限制因 子,HRF)等,使红细胞对补体敏感而溶血。上述3种蛋 白都经糖基化磷脂酰肌醇(GPl)连接在细胞膜上[2]。近 年来,由于各种检测技术的发展使得PNH发病机制的研 究、PNH…     

重型再生障碍性贫血患者免疫抑制治疗后骨髓造血祖细胞水平的变化及其意义

为更好地评价重型再生障碍性贫血(SAA)患者免疫抑制治疗(IST)后骨髓造血功能恢复程度,采用造血祖细胞体外培养技术,动态观察了48例接受IBT的SAA患者及20例正常对照者骨髓晚期红系爆式集落形成单位(mBFU-E)及粒-巨噬系集落形成单位(CFU-GM)的水平变化。结果表明,IST前所有SAA患者骨髓mBFU-E及CFU-GM水平均显著低于正常对照组(P<0.001);IST后1年,29例有效者骨髓mBFU-E及CFU-GM水平显著增高,增高程度与其临床疗效相关;12例mBFU-E及10例CFU-GM水平恢复正常,其中8例患者mBFU-E及CFU-GM水平同时恢复正常。这表明SAA确是一组异质性疾病,其造血功能衰竭与异常免疫关系密切,若去除这种异常,造血功能可获得部分甚至完全重建。     

骨髓增生异常综合征患者骨髓T细胞功能状态研究

目的　研究骨髓增生异常综合征 (myelodysplasticsyndrome ,MDS)患者骨髓中辅助性T淋巴细胞 (Thelper,Th)亚群数量和比例改变及Th1细胞所承担的肿瘤负荷情况。方法　用流式细胞术检测 2 1例MDS患者、18例正常对照和 13例重型再生障碍性贫血患者骨髓单个核细胞胞浆内表达IFN γ的CD4 细胞 (Th1细胞 )和表达IL 4的CD4 细胞 (Th2细胞 )的数量和比例 ;分析MDS患者骨髓原始细胞比例与Th1细胞的相关性。结果　正常对照组骨髓Th1细胞、Th2细胞、Th1细胞 /Th2细胞分别为 (0 .4 8± 0 .10 ) %、(0 .2 4± 0 .19) %、2 .31± 0 .76 ;而MDS患者分别为 :(0 .36± 0 .11) %、(0 .76± 0 .35 ) %、0 .5 1± 0 .13,MDS患者骨髓Th1细胞减少 ,Th1细胞 /Th2细胞比例明显降低 (P <0 .0 1)。重型再生障碍性贫血患者三者指标分别为 (4.75±0 4 9) %、(0 .4 0± 0 .2 8) %、2 6 .5± 8.79,与正常对照组相比较 ,Th1细胞明显增多、Th1细胞 /Th2型细胞比例明显升高 (P <0 0 1) ;随着MDS及MDS转为的AML患者骨髓Th1细胞数量下降 ,白血病转化倾向细胞 ,即原始细胞数量逐渐增多。二者呈负相关 (r =- 0 .5 6 3,P <0 .0 1)。结论　 1.MDS患者T细胞免疫异常 ,Th1细胞 /Th2细胞失衡 ,抗肿瘤细胞免疫中起主要作用的Th1细胞数目减少 ,功