重型再生障碍性贫血患者免疫抑制治疗前后淋巴细胞亚群变化与早期血液学反应的关系

目的:探讨免疫抑制治疗(IST)前后淋巴细胞亚群变化与重型/极重型再生障碍性贫血(SAA/VSAA)患者疾病严重程度及IST后6个月血液学反应(HR)的关系。方法:对2009年12月至2011年10月本中心连续收治的94例行兔抗人胸腺细胞免疫球蛋白(r-ATG)+环孢素A(CsA)治疗的SAA/VSAA患者资料进行回顾性分析,对符合入组条件的26例患者IST前淋巴细胞亚群及细胞因子水平与疾病严重程度、IST前后淋巴细胞亚群及细胞因子变化与IST后6个月HR进行相关性分析。结果:IST前淋巴细胞比例(Lym%)和绝对值计数(Lym#)、各淋巴细胞亚群(包括CD3^+T细胞、CD3^+CD4^+T细胞、CD3^+CD8^+T细胞、CD3^-CD16^+/CD56^+NK细胞和CD19^+B细胞)比例(%)及绝对值(#)、细胞因子(包括IL-1、IL-2、IL-4、IL-6和TNF-α)水平,在SAA和VSAA两组间无统计学差异。IST后3个月和IST后6个月相比较,上述各参数水平无统计学差异。有效组IST后3及6个月Lym%、Lym#、CD3^+T细胞#、CD4^+T细胞%、CD4^+T细胞#、NK细胞%、B细胞#、IL-2水平显著低于治疗前,而无效组IST后3及6个月仅Lym%较治疗前显著降低。IST后3个月,有效组Lym#、CD3^+T细胞#及CD4^+T细胞#显著高于无效组。结论:IST前后淋巴细胞亚群变化所反映的免疫抑制程度,以及治疗后淋巴细胞亚群恢复所反映的免疫重建,可能影响造血恢复和早期血液学反应。免疫重建过程在IST后3月内较为显著。     

普通变异型免疫缺陷病——附二例报告并文献复习

目的 提高对普通变异型免疫缺陷病的认识.方法 报告2例普通变异型免疫缺陷病病例并进行相关文献复习.结果 2例患者均于青少年期发病,表现为反复呼吸道、消化道感染,脾肿大,免疫球蛋白明显减低,可明确诊断为普通变异型免疫缺陷病,例1经脾切除诊断合并脾脏外周T细胞淋巴瘤,例2反复消化道感染、腹泻,并发营养性巨幼细胞性贫血,经静脉注射丙种球蛋白及抗感染对症治疗后,患者临床症状减轻.结论 普通变异型免疫缺陷病起病相对较晚,临床表现多样,以低丙种球蛋白血症和反复呼吸道、消化道感染为特征,早期诊断及正规治疗可改善预后.     

宫内节育器常见副反应及相应处理

我国自1957年既开始研制和临床应用节育器(IUD)，目前它是一种安全有效、经济、简便及可逆性强的避孕方法，一次放置能长期避孕，深受广大育龄妇女的欢迎。但也存在着异常出血、疼痛及感染、嵌顿、子宫穿孔等并发症，尽管采用此法避孕的并发症不高，但实际发生人数却不容忽视。本针对出血，疼痛与感染、嵌顿、子宫穿孔等方面，探讨其原因及防治。     

骨髓增生异常综合征患者有核红细胞糖原染色的意义

Objective To investigate the implications of erythroblasts periodic acid-Schiff (PAS)stain for myelodysplastic syndromes (MDS) dyserythropoiesis, diagnosis and differential diagnosis. Methods PAS stain of bone marrow (BM) erythroblasts in 406 MDS pateints, 207 non-severe aplastic anemia (NSAA), 144 immune thrombocytopenic purpura (ITP), 67 megaloblastic anemia (MegA), 76 iron deficiency anemia (IDA), 50 paroxysmal nocturnal hemoglobinuria (PNH), and 50 acute erythroid leukemia (AEL) as well as some related laboratory parameters in MDS patients were analyzed retrospectively. Results PAS-positive detection rate was significantly higher in MDS (53.0%) than in NSAA (14. 5%), ITP (27.1%) and PNH (16.0%), but was significantly lower in MDS than in AEL (84. 0%) (all P =0.000). There was no significant difference in PAS-positive detection between MDS and MegA (46.3%), or MDS and IDA (40.8 %) (P = 0.310, 0. 052, respectively). Erythroblasts PAS-positive rate (Median, M =1%) and PAS-positive scores (M' =2) was significantly lower in MDS than in AEL (M =8%; M' = 17),and significantly higher than in NSAA(M =0%; M' =0), ITP(M =0%; M' =0), PNH(M =0%; M' =0), MegA (M = 0%; M' = 0), and IDA (M = 0%; M' = 0) (all P ＜ 0.05). The cut-off value of PAS-positive rate and score for distinguishing MDS from the other groups except AEL were 0. 5% and 0. 5, with a sensitivity and specificity of 60.8% and 74.4%, respectively. For MDS patients, the percentage of BM erythroid cells was significantly higher in PAS-positive group than in PAS-negative group (P ＜ 0.05), and so were megakaryocyte count, lymphocyte-like micromegakaryocyte count and percentage of micromegakaryocyte (P =0.002, 0. 000, 0. 000, respectively). HGB、MCV、MCH and MCHC were significantly lower in PASpositive group (all P ＜ 0. 05), and so was the neutrophil alkaling phosphatase (NALP) (P = 0.000). PASpositive detection rate, positive rate and score were higher in MDS patients with abnormal karyotype than with normal karyotype, and were also higher in IPSS high/intermediate-risk 2 group than in low/intermidiate-risk 1group. Conclusion The positive reaction of erythroblasts PAS stain is an indicator of dyserythropoiesis. It is helpful to the diagnosis of MDS patients.     

再生障碍性贫血患者接受免疫抑制治疗发生致死性CMV肺炎一例报告并文献复习

巨细胞病毒感染( Cytomegalovirus infection,CMVI)和CMV病(Cytomegalovirus disease,CMVD)是接受造血干细胞移植和实体器官移植患者的常见并发症和重要死亡原因.近年,随着新型免疫抑制剂的应用,非移植治疗患者发生CMV活化的机会也明显增加,但迄今尚无重型再生障碍性贫血(SAA)患者接受免疫抑制治疗(IST)而罹患致死性CMVI的报告.我们收治1例SAA患者采用兔源抗胸腺细胞球蛋白(r-ATG)联合环孢素(CsA)治疗,患者发生严重CMVI并死亡,现报告如下.     

AME（P）方案治疗成人难治及复发急性白血病13例

ＡＭＥ（Ｐ）方案治疗成人难治及复发急性白血病１３例张凤奎，崔亚强，梁晋全，葛雪茹急性白血病（ＡＬ）治疗失败常被归因于耐药的产生，因而治疗难治及复发ＡＬ应选用新的有效药物或进行新的药物组合。我们采用阿糖胞苷（Ａ），米托蒽醌（Ｍ），足叶乙甙（Ｅ）及泼尼松...     

LDH·RA方案治疗急性早幼粒细胞白血病

我们对３０例急性早幼粒细胞白血病患者采用ＬＤＨ·ＲＡ方案（Ｈ＊０．５～１ｍｇ／ｄ，ＲＡ４０～６０ｍｇ／ｄ）诱导缓解治疗，完全缓解（ＣＲ）２７例（９０％）、部分缓解１例，总有效率９３．３％。有关缓解指数：外周血早幼粒细胞消失中位天数２７．７（２２～５２）天，骨髓缓解中位天数３７．５（２５～６８）天，取得ＣＲ中位天数３９．５天。缓解后采用早期强化、巩固治疗，ＨＡ、ＤＡ、ＥＡ、ＨＤ－Ａｒａ－Ｃ四种方案交替，联合化疗间歇期口服ＲＡ２０～４０ｍｇ／ｄ，或（和）Ｈ１ｍｇ／ｄ肌注，缓解病例中有１６例仍维持缓解状态，最长已达４４个月。     

多发性骨髓瘤继发急性非淋巴细胞性白血病M2型一例

患者，女，63岁。2002年2月以全身骨痛半年伴乏力1周住院。查体：T36．5℃，Bp130／70mmHg（1mmHg=0.133kPa），皮肤未见出血点，浅表淋巴结无肿大。胸骨压痛。两肺呼吸音清，心率75次/min，律齐。腹软，肝脾未及。双下肢无水肿。T10、T12、S1椎体及棘突压痛。     

多中心Castleman病临床病理分析

目的：分析多中心Castleman病(MCD)的临床病理特点。方法：MCD患者5例,其中浆细胞型(PC)3例,透明血管型(Hv)2例,均为男性,年龄43岁～62岁(中住年龄50岁)。4例接受COP化疗。并分别应用糖皮质激素、沙利度胺 干扰素维持,1例放弃治疗。结果：临床情况5例分别不同程度表现发热、乏力、盗汗等非特异性全身症状,5例均存在多部位浅表淋巴结肿大,3例脾脏肿大。实验室结果：出现贫血及肾功能损害各有3例,4例患者出现多克隆高1球蛋白血症,4例患者骨髓浆细胞或嗜酸粒细胞增多。治疗转归：1例4疗程COP治疗后生存8a,2例治疗后达PR,带病生存4个月、8个月,仍随访中。结论：多中心CD常累及多系统损伤,且表现较淋巴结肿大为早,PC型全身症状相对较重,致使MCD早期诊断存在困难,确诊须经淋巴结病理活检证实。     

骨髓增生异常综合征患者有核红细胞糖原染色的意义

Objective To investigate the implications of erythroblasts periodic acid-Schiff (PAS)stain for myelodysplastic syndromes (MDS) dyserythropoiesis, diagnosis and differential diagnosis. Methods PAS stain of bone marrow (BM) erythroblasts in 406 MDS pateints, 207 non-severe aplastic anemia (NSAA), 144 immune thrombocytopenic purpura (ITP), 67 megaloblastic anemia (MegA), 76 iron deficiency anemia (IDA), 50 paroxysmal nocturnal hemoglobinuria (PNH), and 50 acute erythroid leukemia (AEL) as well as some related laboratory parameters in MDS patients were analyzed retrospectively. Results PAS-positive detection rate was significantly higher in MDS (53.0%) than in NSAA (14. 5%), ITP (27.1%) and PNH (16.0%), but was significantly lower in MDS than in AEL (84. 0%) (all P =0.000). There was no significant difference in PAS-positive detection between MDS and MegA (46.3%), or MDS and IDA (40.8 %) (P = 0.310, 0. 052, respectively). Erythroblasts PAS-positive rate (Median, M =1%) and PAS-positive scores (M' =2) was significantly lower in MDS than in AEL (M =8%; M' = 17),and significantly higher than in NSAA(M =0%; M' =0), ITP(M =0%; M' =0), PNH(M =0%; M' =0), MegA (M = 0%; M' = 0), and IDA (M = 0%; M' = 0) (all P ＜ 0.05). The cut-off value of PAS-positive rate and score for distinguishing MDS from the other groups except AEL were 0. 5% and 0. 5, with a sensitivity and specificity of 60.8% and 74.4%, respectively. For MDS patients, the percentage of BM erythroid cells was significantly higher in PAS-positive group than in PAS-negative group (P ＜ 0.05), and so were megakaryocyte count, lymphocyte-like micromegakaryocyte count and percentage of micromegakaryocyte (P =0.002, 0. 000, 0. 000, respectively). HGB、MCV、MCH and MCHC were significantly lower in PASpositive group (all P ＜ 0. 05), and so was the neutrophil alkaling phosphatase (NALP) (P = 0.000). PASpositive detection rate, positive rate and score were higher in MDS patients with abnormal karyotype than with normal karyotype, and were also higher in IPSS high/intermediate-risk 2 group than in low/intermidiate-risk 1group. Conclusion The positive reaction of erythroblasts PAS stain is an indicator of dyserythropoiesis. It is helpful to the diagnosis of MDS patients.