小剂量马法兰治疗中、高危骨髓增生异常综合征临床疗效观察

目的 观察小剂量马法兰治疗中、高危骨髓增生异常综合征(MDS)的疗效和药物不良反应.方法 共有按MDS国际预后积分系统(IPSS)判断为中、高危组的30例初治患者入组.治疗方案为:马法兰2 mg,口服,每日1次,直至病程进展所致全血细胞减少加剧或骨髓原始细胞比例增高停药.达完全缓解(CR)或部分缓解(PR)者继续用药直至病情复发停药.结果 按2006年MDS 国际工作组(IWG)疗效修订标准判定,CR 9例(30.0%),PR 3例(10.0%),骨髓缓解(MCR)加血液学进步(HI)3例(10.0%),MCR 1例(3.3%),病情稳定4例(13.3%),治疗失败10例(33.3%).总有效率66.7%.骨髓增生活跃及以下组患者的CR+PR率(60.0%)和总有效率(80.0%)明显高于骨髓增生明显活跃及以上组(分别为0%和40%)(P值分别为0.002和0.045).中位随访时间为15.5(1～52)个月,中位生存时间为18个月,中位无复发生存时间为11个月.治疗过程中3例患者出现轻度骨髓抑制,1例因血小板输注无效导致脑出血而死亡,未见其他明显药物不良反应.结论 小剂量马法兰是治疗中、高危MDS患者的一种安全、有效的方法,并且可能更适用于骨髓增生低下的患者.     

小剂量马法兰治疗中、高危骨髓增生异常综合征临床疗效观察

目的 观察小剂量马法兰治疗中、高危骨髓增生异常综合征(MDS)的疗效和药物不良反应.方法 共有按MDS国际预后积分系统(IPSS)判断为中、高危组的30例初治患者入组.治疗方案为:马法兰2 mg,口服,每日1次,直至病程进展所致全血细胞减少加剧或骨髓原始细胞比例增高停药.达完全缓解(CR)或部分缓解(PR)者继续用药直至病情复发停药.结果 按2006年MDS 国际工作组(IWG)疗效修订标准判定,CR 9例(30.0%),PR 3例(10.0%),骨髓缓解(MCR)加血液学进步(HI)3例(10.0%),MCR 1例(3.3%),病情稳定4例(13.3%),治疗失败10例(33.3%).总有效率66.7%.骨髓增生活跃及以下组患者的CR+PR率(60.0%)和总有效率(80.0%)明显高于骨髓增生明显活跃及以上组(分别为0%和40%)(P值分别为0.002和0.045).中位随访时间为15.5(1～52)个月,中位生存时间为18个月,中位无复发生存时间为11个月.治疗过程中3例患者出现轻度骨髓抑制,1例因血小板输注无效导致脑出血而死亡,未见其他明显药物不良反应.结论 小剂量马法兰是治疗中、高危MDS患者的一种安全、有效的方法,并且可能更适用于骨髓增生低下的患者.     

拓扑替康治疗恶性血液病研究现况

拓扑异构酶Ⅰ抑制剂拓扑替康单独或联合其它化疗药物是治疗骨髓增生异常综合征等恶性血液病的新的有效方案。本文对拓扑替康作用机制、临床疗效及其毒副作用作一综述。     

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.     

亚标准剂量静脉丙种球蛋白治疗特发性血小板减少性紫癜临床观察

198 1年Ｉｍｂａｃｈ首次证明大剂量静脉丙种球蛋白(ＨＤ ＩＶＩＧ)治疗特发性血小板减少性紫癜 (ＩＴＰ)有效[1 ] 。由于疗效显著 ,ＩＶＩＧ现已成为ＩＴＰ治疗的重要药物 ,使用方法为0 .4ｇ·ｋｇ- 1 ·ｄ- 1 × 5ｄ或 1ｇ·ｋｇ- 1 ·ｄ- 1 × 2ｄ[2 ] ,总剂量 2ｇ ｋｇ为其标准用量。由于ＩＶＩＧ费用昂贵 ,我们对较小剂量的ＩＶＩＧ治疗ＩＴＰ的疗效进行了临床观察 ,证实亚标准剂量静脉丙种球蛋白 (Ｓｕｂ ＨＤ ＩＶＩＧ ,0 .2ｇ·ｋｇ- 1 ·ｄ- 1 × 5ｄ)组与标准ＨＤ ＩＶＩＧ对照组的疗效差异无显著性。病例和方法1　病例选择和分组…     

利妥昔单抗净化动员B细胞非霍奇金淋巴瘤外周血干细胞的初步研究

 目的 观察利妥昔单克隆抗体（Rituximab,利妥昔单抗）对B细胞非霍奇金淋巴瘤（NHL）患者外周血干细胞（PBSCs）净化动员的作用。方法 8例CD+20 B细胞NHL患者,在应用CHOP类化疗±利妥昔单抗4～6疗程诱导／巩固治疗后进行大剂量环磷酰胺（HD-CTX）+粒细胞集落刺激因子（G-CSF）联合利妥昔单抗（375 mg·m-2·d-1,第-1,7天）体内净化动员PBSCs。观察利妥昔单抗副反应、骨髓抑制期及相关并发症、PBSCs采集时间、数量以及采集物肿瘤标志物基因等。结果 在外周血干细胞动员过程中仅1例患者发生轻度利妥昔单抗相关的皮疹。PBSCs平均采集时间为CTX应用后（11.6±1.0）d,中位采集次数2（1～3）次。采集物平均单个核细胞（MNC）（3.4±1.0）×108／kg,平均CD+34细胞数（3.6±1.7）×106／kg。5例完成移植患者中3例移植后IgH／TCR转为阴性,1例治疗前后均为阴性,1例早期复发。4例无病生存。结论 利妥昔单抗不影响B细胞NHL患者PBSCs的动员效果,安全性好,并能加强体内净化作用。     

唑来膦酸钠致多发性骨髓瘤患者下颌骨坏死一例

患者,男,43岁.因乏力、腰酸2个月,腰痛1个月于2005年8月入我院.     

反应停加联合化疗治疗浆细胞白血病一例

浆细胞白血病 (ＰＬ)是一种少见的恶性浆细胞疾病 ,常规治疗疗效差 ,最近我们采用反应停 +联合化疗成功治疗 1例原发性ＰＬ(ＰＣＬ) ,现报告如下。患者 ,男 ,5 6岁。因乏力半年伴发热 4ｄ ,于 2 0 0 0年 6月2日入院。查体 :体温 38.2℃ ,重度贫血貌 ,脾肋缘下 2 .0ｃｍ ,余无明显阳性体征。Ｈｇ 5 2ｇ/Ｌ ,ＷＢＣ 5 .5 4× 10 9/Ｌ ,浆细胞 0 4 4 ,浆细胞绝对计数为 2 .4 4× 10 9/Ｌ ,ＢＰＣ 5 4× 10 9/Ｌ ;骨髓涂片浆细胞比例为 0 .70 5。骨髓单个核细胞流式细胞术免疫分型为ＣＤ9+ 、ＣＤ19-、ＣＤ2 0 -、ＣＤ3 8+ 、ＤＲ-,染色体核型…     

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.     

盐酸雷莫司琼口腔崩解片预防恶性血液疾病联合化疗所致的胃肠反应

目的：观察盐酸雷莫司琼口腔崩解片预防恶性血液疾病患者联合化疗所致胃肠反应的疗效和安全性。方法：采用随机、对照方法，将入选病例随机分为治疗组（含服盐酸雷莫司琼口腔崩解片0．1mg）和对照组（静脉注射盐酸托烷司琼5mg）。结果：可评价疗效及安全性病例110例。两种药物对控制化疗所致食欲缺乏、恶心和呕吐的有效率相似。第1～7天盐酸雷莫司琼对食欲缺乏的完全控制率（52．7％～63．6％）、第4～7天对恶心的完全控制率（43．6％～56．4％）及第3、4、6天对呕吐的完全控制率（80．0％、87．3％和90．9％）明显优于盐酸托烷司琼注射剂，差异有显著性（P〈0．05）。盐酸雷莫司琼不良反应轻，主要为便秘、头痛、体热感和疲倦，为一过性，其发生率与盐酸托烷司琼注射剂相似。结论：盐酸雷莫司琼口腔崩解片能有效预防恶性血液病患者联合化疗所致的胃肠反应，适用于因各种原因不能吞服药片的患者。