分化诱导剂联合雄激素或小剂量化疗药物治疗骨髓增生异常综合征

报道３６例骨髓增生异常综合征的治疗效果，原始细胞不增多的ＭＤＳ（ＲＡ和ＲＡＳ）１７例，以雄激素丙酸睾丸素或康力龙联合分化诱导剂全反式维甲酸和１，２５（ＯＨ）２Ｄ３治疗，部分病例尚加用小剂量强的松；原始细胞增多的ＭＤＳ（ＲＡＥＢ、ＣＭＭＬ及ＲＡＥＢＴ）１９例，以小剂量阿糖胞苷或／和小剂量阿克拉霉素化疗并联用分化诱导剂，前组的有效率为７０．６％，基本缓解率为２９．４％，后组的有效率为５７．９％，完全缓     

骨髓增生异常综合征诊断与分期的探讨──附116例临床分析

报告ＭＤＳ１６６例，其中ＲＡ１２２例（７３．５％），ＲＡＳＳ例（３％）’ＲＡＥＢ２５例（１５％），ＣＭＭＬ１例（０．６％），ＲＡＥＢ－Ｔ１３例（７．９％）．并对其诊断标准，分期和治疗等进行了讨论。Ｂｅｎｎｅｔｔ改良的ＦＡＢ诊断与分型标准已被广泛应用，本文虽按此标准进行统计，但本文认为：①ＦＡＢ的５个亚型不是独立的，而是同一疾病病程发展的不同阶段；②ＣＭＭＬ实际为白血病，不宜划入ＭＤＳ范畴；③ＲＡＳ系难治性贫血伴环形铁粒幼细胞增多，同原发性铁粒幼细胞贫血有别，可划入ＲＡＥＢ或ＲＡＥＢ－Ｔ期内；④骨髓片中出现原始细胞丛或群应看成向白血病发展的表现；⑤血细胞的病态造血是诊断本病的关键；⑥治疗上用单一分化诱导剂的疗效低，采用联合应用诱导分化剂有明显协调作用。     

骨髓增生异常综合征诊断与分期的探讨：附116例临床分析

报告ＭＤＳ１６６例，其中ＲＡ１２２例（７３．５％），ＲＡＳ５例（３％），ＲＡＥＢ２５例（１５％），ＣＭＭＬ１例（０．６％），ＲＡＥＢ－Ｔ１３例（７．９％）。并对其诊断标准，分期和治疗等进行了讨论。Ｂｅｎｎｅｔｔ改良的ＦＡＢ诊断与分型标准已被广泛应用，本文虽按此标准进行统计，但本文认为：①ＦＡＢ的５个亚型不是独立的，而是同一疾病病程发展的不同阶段；②ＣＭＭＬ实际为白血病，不宜划入ＭＤＳ范畴；③ＲＡＳ     

小剂量化疗药加与不加GM-CSF治疗高危MDS6例

小剂量化疗药加与不加ＧＭ－ＣＳＦ治疗高危ＭＤＳ６例赵文理候冰王歧山李梅生高危性骨髓增生异常综合征（ＭＤＳ）是指原始细胞增多，如不治疗终将转变为白血病的一组病例，主要包括分型中的ＭＤＳ－ＲＡＥＢ（难治性贫血伴原始细胞增多）及ＭＤＳ－ＲＡＥＢＴ（转变中的...     

COMP和PBM化疗方案治疗晚期鼻咽癌的疗效分析

我们以ＣＴＸ＋ＶＣＲ＋ＭＴＸ＋ＤＤＰ（ＣＯＭＰ）和ＤＤＰ＋ＢＬＭ＋ＭＴＸ（ＰＢＭ）两种化疗方案治疗复发和转移的晚期鼻咽癌５８例。４９例可评价客观疗效。结果ＣＯＭＰ组取得ＣＲ３例、ＰＲ９例，有效率（ＣＲ＋ＰＲ）为５２．２％；ＰＢＭ组ＣＲ１例、ＰＲ９例，有效率（ＣＲ＋ＰＲ）为３８．５％。两者无显著性差异（Ｐ＞０．０５）．治疗后有效病例缓解期为５～５６月，中位缓解期为２５月。两种方案对鼻咽癌肺转移均较好，对肝转移疗效最差。ＣＯＭＰ方案对鼻咽癌远处转移疗效较好，对肺转移有效率为５８．３％（７／１２）．可作为鼻咽癌远处转移尤其是肺转移的首选。ＰＢＭ方案对鼻咽癌原灶复发疗效较好，对鼻咽癌复发有效率为４２．９％（３／７），可作为鼻咽癌原灶复发或放射治疗后辅助化疗首选方案。     

COMP与PBM化疗方案治疗晚期鼻咽癌的疗效分析

我们以ＣＴＸ＋ＶＣＲ＋ＭＴＸ＋ＤＤＰ（ＣＯＭＰ）和ＤＤＰ＋ＢＬＭ＋ＭＴＸ（ＰＢＭ）两种化疗方案治疗复发和转移的晚期鼻咽癌５８例，４９例可评价客观疗效。结果ＣＯＭＰ组取得了ＣＲ３例，ＰＲ９例，有效率（ＣＲ＋ＰＲ）为５２．２％；ＰＢＭ组ＣＲ１例，ＰＲ９例，有效率（ＣＲ＋ＰＲ）为３８．５％。两者无显著性差异（Ｐ〉０．０５）。治疗后有效病例缓解期为５～５６月，中位缓解期为２５月。两种方案对鼻咽癌肺转移均较     

成人急性白血病强化治疗67例预后分析

目的：观察成人急性白血病（ＡＬ） 患者完全缓解（ＣＲ） 后强化治疗的效果。方法：对６７ 例ＣＲ后的成人ＡＬ患者进行强化治疗,急性髓细胞性白血病（ＡＭＬ）以中剂量阿糖胞苷（ＩＤ－ Ａｒａ－ Ｃ）方案为主,急性淋巴细胞性白血病（ＡＬＬ）以中剂量氨甲蝶呤（ＩＤ－ ＭＴＸ）方案为主。结果：４８ 例ＡＭＬ患者中位ＣＲ 期１６ 个月,预期３ 年和４ 年无病生存（ＤＦＳ）为３６９％ 和２１１ ％ ;２３ 例（４７９ ％） 患者复发。１９ 例ＡＬＬ 患者中位ＣＲ 期１４ 个月,预期４ 年ＤＦＳ 为３１５％ ;１０ 例（５２６％ ） 患者复发。结论：以ＩＤ－ Ａｒａ－ Ｃ 为主的强化方案及以ＩＤ－ ＭＴＸ 为主的强化方案分别能延长ＡＭＬ及ＡＬＬ患者的ＤＦＳ,降低复发率     

人胎肝抑瘤物体外净化自体骨髓治疗急性白血病13例报告

用人类胎肝低分子天然抑瘤物（ＬＭＷ－ＮＴＳ）对１３例缓解期（ＣＲ）急性白血病休外净化后自体骨髓移植（ＡＢＭＴ），其中ＡＬＬ４例（ＣＲ１３例，ＣＲ２１例），ＡＮＬＬ９例（ＣＲ１７例，ＣＲ２２例）。１２例获造血重建，１例因霉菌败血症早期死亡。移植后持续缓解８例，缓解时间＞１２个月７例，＞６个月１例。４例移植后３月～６月复发。木文对该净化方法及ＡＢＭＴ近期疗效进行讨论。     

小剂量HA方案治疗急性髓系白血病和骨髓增生异常综合征的临？…

本文通过回顾性研究对小剂量ＨＡ方案［高三尖杉（Ｈｏｍ）加阿糖胞苷（Ａｒａ－Ｃ）］在急性髓细胞系白血病（ＡＭＬ）和骨髓增生异常综合征（ＭＤＳ－ＲＡＥＢＴ）治疗中的作用进行了总结。１临床资料 自１９９１年起我科ＡＭＬ和ＭＤＳ患者共４４例接受了小剂量ＨＡ方案治疗，其中初治患者２７例，复发患者１７例。初治组：男１８例，女９例，中数年龄４９岁，＞６０岁者占３３．３％，＞５０岁者为３７％；复发组：男 １０例，女 ７例，中数年龄 ４８岁，＞ ５０岁者占４１．２％，＞６０岁为５．９％。化疗方案：Ｈｏｍ １ｍｇ／日 ｔ…     

含顺铂,平阳霉素联合化疗治疗食管癌：（附166例疗效分析）

从１９８３年１１月－１９８９年８月，采用以顺铂、平阳霉素为主，分别与Ｅｔｏｐｏｓｉｄｅ（Ｖｐ－１６），ＶＣＲ，ＭＭＣ，Ｎｉｔｒｏｃａｐｈａｎｅ（ＡＴ－１２５８）结合的ＤＥＰ、ＤＶＰ、ＤＭＰ、ＤＮＰ４种联合化疗方案，随机分组治疗晚期食管癌１６６例，完全缓解（ＣＲ）１４例（８．４％），部分缓解（ＰＲ）７１例（４２．８％），稍效（ＭＲ）４３例（２５．９％），无效（ＮＲ）３８例（２２．９％），有效率（ＣＲ     

Expression of p53, bcl-2 and ras oncoproteins and apoptosis levels in acute leukaemias and myelodysplastic syndromes.

We analysed by immunocytochemistry the expression of p53, bcl-2 and ras proteins in bone marrow blasts from 59 patients with acute leukaemia (AL), 36 myeloid (AML) and 23 lymphoid (ALL), and from 22 patients with myelodysplastic syndrome (MDS); our aim was to examine if abnormalities in their expression were associated with peculiar biological and clinical findings, or with an altered apoptosis rate, as measured by TUNEL technique. The oncoproteins were expressed with extreme variability, without significant differences among the various morphological or immunological AL subtypes. The mean percentages of bcl-2+ blasts were significantly higher in AML than in MDS (p = 0.01), and in MDS with bone marrow blastosis than in the forms without excess of blasts (p = 0.007). The lowest percentages of apoptotic cells were observed in ALL (mean 1%, p = 0.006), whereas in MDS the apoptotic index was higher (16.7%) than in AML (8.6%) and than in the normal controls (10.8%). but the difference tended to be statistically significant only for cases of refractory anaemia. Whereas in AML and MDS the apoptotic rate was independent of the oncoprotein expression, in ALL there was a significant linear relationship between TUNEL and ras positivity (p = 0.01). Among AML patients treated with intensive polychemotherapy, no differences were observed in oncoprotein expression and apoptotic rate between responders and resistant cases. In conclusion, our data are in agreement with the hypothesis that decreased apoptosis and enhanced cell survival are associated with AL, whereas a high level of apoptosis may be responsible for the ineffective hematopoiesis in MDS; abnormal expression of oncoproteins, even if not strictly related to apoptosis level, may influence disease behaviour.     

Treatment options in myelodysplastic syndromes: a new frontier

Myelodysplastic syndromes (MDS) are very heterogeneous diseases in terms of clinical presentation and prognosis. Patients with pure red cell dysplasias have a life expectancy of more than 10 years, whereas those with refractory anemias with excess blasts have survival shorter than 6 months. Until a few years ago, therapeutic options were palliative and supportive care only. Quite recently, the treatment panorama for MDS has radically changed and the different biological behavior of MDS requires a precise choice among completely different therapies: immunosuppressive agents, anti-apoptotic agents and growth factors are effective in low risk MDS, whereas epigenetic drugs, tyrosine kinase inhibitors, and possibly high dose chemotherapy and bone marrow transplantation are valuable in high risk MDS. We review the results of such therapies and the selection criteria for MDS patients.     

Expression of Lung Resistance Protein and Correlation with Other Drug Resistance Proteins and Outcome in Myelodysplastic Syndromes

The major vault lung resistance protein LRP is a cytoplasmic protein involved in drug resistance, especially in acute myeloid leukemia. We looked for LRP overexpression, using immunocytochemistry with LRP 56 monoclonal antibody, on marrow slides from 41 cases of myelodysplastic syndromes (MDS). LRP overexpression (LRP+) was defined by expression of LRP 56 in at least 20% of marrow blasts. LRP overexpression was seen in 19 (46%) cases. Concordant results between LRP overexpression and P-glycoprotein (PGP) expression were seen in 66% of the cases (p = 0.03), and discordant results (LRP+ and PGP-, or LRP-and PGP+) in 33% of the cases. No correlation was seen between LRP overexpression and FAB type, karyotype, CD34, p53 expression and bc12 overexpression in blasts. Furthermore, in the 18 cases treated with anthracycline-AraC intensive chemotherapy and the 7 cases treated with low dose AraC, the response rate was not significantly different in LRP+ and LRP-patients. Survival was also similar in LRP+ and LRP—patients. In conclusion, LRP overexpression is probably more frequent in MDS than in de novo AML and, as in AML, is only partially correlated with PGP expression. In our experience, however, LRP was not a prognostic factor for response to chemotherapy and survival in MDS.     

Treatment Options in Myelodysplastic Syndromes: A New Frontier

Abstract

Myelodysplastic syndromes (MDS) are very heterogeneous diseases in terms of clinical presentation and prognosis. Patients with pure red cell dysplasias have a life expectancy of more than 10 years, whereas those with refractory anemias with excess blasts have survival shorter than 6 months. Until a few years ago, therapeutic options were palliative and supportive care only. Quite recently, the treatment panorama for MDS has radically changed and the different biological behavior of MDS requires a precise choice among completely different therapies: immunosuppressive agents, antiapoptotic agents and growth factors are effective in low risk MDS, whereas epigenetic drugs, tyrosine kinase inhibitors, and possibly high dose chemotherapy and bone marrow transplantation are valuable in high risk MDS. We review the results of such therapies and the selection criteria for MDS patients.     

Cytogenetic abnormalities in Tunisian de novo myelodysplastic syndrome: a comparison with other populations

Cytogenetic analysis was performed on 224 bone marrow (BM) of Tunisian patients with de novo myelodysplastic syndrome (MDS) at our institution from January 1993 to December 2006.According to French–American–British (FAB) criteria, there were 36% of patients with refractory anaemia (RA), 26% with refractory anaemia with excess of blasts (RAEB), 10% with refractory anaemia with ringed sideroblasts (RARS), 12% with chronic myelomonocytic leukaemia (CMML), 9% refractory anaemia with excess of blasts in transformation (RAEB-t) and 7% of unclassified MDS. A clonal chromosomal abnormality was observed in 51% of the patients. The most frequent karyotypic change was 5q− in 30 cases (13%), followed by −7/7q− in 17 cases (8%), del(12p) in 8 cases (4%), del(20q) and trisomy 8 in 7 cases each (3%), i(17q) in 2 cases (1%) and −y in only one case (0.4%).This is the first large comparative series of MDS from an Arab country, with cytogenetic analysis showing haematological and cytogenetic features similar to those of MDS population of European or mixed European-subsaharian African origin (like Brazil), but different from those seen in Eastern populations.     

Myelodysplastic syndrome or acute myeloid leukemia? A study of 28 cases presenting with borderline features

Some patients present borderline features between acute myeloid leukemia (AML) and typical myelodysplastic syndromes (MDS): an excess of blasts insufficient to conclusively diagnose AML, yet above the figures usually compatible with MDS or the presence of Auer rods associated with a moderate excess of blasts. This presents considerable difficulties in diagnosis and management. The authors studied 28 such cases using the French-American-British Co-operative Group (FAB) classification, which groups them into a separate category termed "refractory anemia with excess of blasts in transformation" (RAEB-T). This was found to be a heterogenous group. Certain patients (4/28) had a previously established myelodysplasia, but most presented directly as RAEB-T. Two very different pictures emerged: a few patients (4/28) were young, with presentation and evolution similar to classic AML, for whom combination chemotherapy was effective; the majority (20/28) were older, with more varied clinical and cytologic presentation, for whom chemotherapy was of little effect and who presented a picture resembling classic RAEB with a median survival of 10 months.     

Cytogenetic characteristics of 665 patients with myelodysplastic syndrome in China: A single-center report

The karyotype is highly important for diagnosis and prognosis in myelodysplastic syndrome (MDS). The objective of the present study was to investigate the cytogenetic characteristics of patients with MDS in China. The karyotypes of 665 Chinese patients with MDS were analyzed, and it was identified that 298 cases (298/665, 44.8%) had abnormal karyotypes. Among the 298 patients with abnormal karyotypes, the 75 patients with trisomy 8 (+8) constituted the most common subset (75/298, 25.2%). The incidence of abnormal karyotypes was significantly higher in patients who were ≥51 years old compared with those <51 years old, (54.8 vs. 34.7%, respectively; P<0.05). Based on World Health Organization (WHO) classification-based Prognostic Scoring System (WPSS) criteria, the incidence of poor-prognosis karyotypes was significantly higher (17.4 vs. 5.4%; P<0.05) in the older patient group, and based on the Revised International Prognostic Scoring System (IPSS-R) criteria, the incidence of poor-/very poor-prognosis karyotypes was also significantly higher (17.4 vs. 6.6%; P<0.05) in patients ≥51 years old compared with younger ones. Based on the WHO classification of MDS subtypes, the incidence of abnormal karyotypes in patients with high percentages of bone marrow (BM) blasts [excess blasts (EB)-I + EB-II, ≥5% blasts] was significantly higher than that in patients with low percentages of BM blasts (those with single lineage dysplasia + multilineage dysplasia, <5% blasts) (62.5 vs. 36.0%; P<0.05). The incidence of poor-prognosis karyotypes based on WPSS criteria was significantly higher in patients with high percentages of BM blasts than those with low percentages (22.0 vs. 6.9%, respectively; P<0.05), and the incidence of poor-/very poor-prognosis karyotypes based on IPSS-R criteria was also significantly higher (23.0 vs. 7.4%, respectively; P<0.05). These results demonstrate that +8 is the most common abnormal karyotype in Chinese patients with MDS. Age and the percentage of BM blasts are associated with the incidence of both abnormal karyotypes and karyotypes with poor prognosis. The results of cytogenetic abnormalities in this study will supplement the data on patients of MDS in China.     

Interleukin-2 Therapy for Myelodysplastic Syndrome: Does It Work?

Recent clinical studies suggested that interleukin-2 (IL-2) has therapeutic potential for some hematologic malignancies, but the therapeutic role of IL-2 for myelodysplastic syndrome (MDS) is still unclear. MDS is a clonal malignant disorder which often involves a variety of immunologic abnormalities. Examination of the effects of IL-2 on MDS in vitro yielded the following results: (1) IL-2 did not induce the proliferation of blasts in most MDS cases. (2) The cytotoxicity of IL-2-induced lymphokine-activated killer (LAK) cells for cell lines and MDS blasts was reduced in the high-risk MDS group (refractory anemia with excess blasts (RAEB), RAEB in transformation and MDS transformed to acute leukemia), but it was still preserved in the low-risk MDS group (refractory anemia (RA) and RA with ringed sideroblasts). However, considerable variation in LAK cell cytotoxicity was noted in each group. (3) The reduced LAK cell cytotoxicity observed in MDS was explained, at least in part, by the presence of a reduced of number of natural killer (NK) cells amongst the LAK cells. (4) MDS patients who have a high blood soluble IL-2 receptor (sIL-2R) level often had defects in NK and CD8+ T cells. These in vitro findings suggest that the response to IL-2 is heterogeneous in MDS patients, and those who have a low-risk MDS subtype and/or a low blood sIL-2R level, may be prone to respond to IL-2 therapy. Clinical trials are mandatory in order to elucidate the efficacy of IL-2 therapy in the treatment of MDS.     

Low Dose Cytosine Arabinoside Therapy in Myelodysplastic Syndrome and Acute Myeloid Leukemia

Four patients with acute myeloid leukemia (AML) and three withmyelodysplastic syndrome (MDS) were given low dose cytosinearabinoside (Ara-C) therapy. One patient with de novo AML andtwo patients having refractory anemia with excess of blasts(RAEB) achieved responses. Although the responses lasted foronly a short duration (2–3 months), the therapy was welltolerated and not accompanied by severe complications, whilesevere cytopenia was a frequent side effect with transfusionsbeing necessary in most patients. This therapy could be clinicallyeffective for certain types of AML and MDS (especially RAEBand RAEB in transformation).