地西他滨治疗慢性粒-单核细胞白血病转化的急性白血病一例

患者,男,68岁,因间断发热2年于2009年6月25日入我院风湿免疫科,体温波动于38～40 ℃.入院查体:体温36.7 ℃,脉率82次/min,呼吸16次/min,血压130/80 mm Hg(1 mm Hg=0.133 kPa).轻度贫血貌,全身浅表淋巴结未触及肿大,心音有力,未闻及病理性杂音,肝脾肋缘下未及.     

慢性粒-单核细胞白血病并发食管癌一例

患者男，62岁。2005年4月因间断发热、咳嗽、全身酸痛2年入院。2年前因反复发热38℃～39℃，血象异常，于外院查血象白细胞2．5×10^9／L、红细胞2．66×10^12／L，单核细胞39％。骨髓穿刺结果：原粒5．5％，早幼粒1．5％，幼稚单核2．5％，单核11％，巨核细胞38个，多为小巨核。粒系统易见Pelger核型。红系减少，粒：红=7．0：1。给予叶酸、维生素B小速力菲疗效不佳。此次入院体格检查：T39．5℃、消瘦、贫血貌，浅表淋巴结不大，胸骨无压痛，脾大于左肋下1．5cm。血常规：白细胞6．4×10^9／L，红细胞2．68×10^12／L，     

小剂量鬼臼乙叉甙治疗慢性粒-单核细胞白血病

作者于1983-1984年用鬼臼乙叉甙治疗了10例慢性粒-单核细胞白血病(CMML).鬼臼乙叉甙100mg/天,连服3天,即足以达到降低白细胞计数,消除血浆渗出.而对于非急需病人可采用50mg/次1周2次,无论是作为维持剂量还是首次治疗都有效.10例病人中,7例获得了持久临床疗效.2例有心包腔及胸腔积液的病人疗效显著,治疗1周后即缓解.对于单核细胞皮肤浸润的病人也获得了同样的疗效,血中或骨髓中原始细胞增加的病人疗效较差.接受治疗的10例病人中只有2例产生了轻度脱发,1例有轻度的胃肠道反应.另一例白细胞计数迅速增加并伴有胸膜及心包渗出的CMML病人应用羟基脲无效,应用丙亚胺疗效不明显,采用鬼臼乙     

慢性粒-单核细胞白血病分型探讨--附24例分析

本文对24例慢性粒-单核细胞白血病(CMML)的临床特征和血液学表现进行分析.根据骨髓粒单核细胞系和红细胞系比例、血细胞病态造血、外周血白细胞计数以及脾脏肿大程度等特征,将CMML分为两种亚型,即具有骨髓增殖性疾病(MPD)特征的CMML为真性CMML,应归于MPD或慢性髓细胞白血病(CML)的一个亚型;具有骨髓增生异常综合征(MDS)特征的CMML为MDS-CMML,称单核细胞增多的难治性贫血(RAM).同时将这两种亚型的诊断标准进行提议.     

慢性粒-单核细胞白血病的11号染色体三体

目前仅有少数有关慢性粒-单核细胞白血病(CMML)染色体研究的资料,且未证实任何特异性的异常。本文首次报道两例CMML有11号染色体三体(+11)的异常。例1,男性,67岁,因白细胞增多和贫血而就诊。体检示肝、脾和淋巴结不肿大。白细胞总数15.3×10~9/L,其中单核细胞占39%。其2-萘酚丁酸酯酶染色呈阳性反应且可为氟化钠所抑制。血清溶菌酶77.4μg/ml。确诊后第5年患者发生急变,给予大剂量阿糖胞苷治疗,未获缓解。例2,男性,56岁,因白细胞增多和贫血入院。体检示肝脾明显肿大。白细胞总数51.3×10~9/L,其中     

急性粒-单核细胞白血病缓解五年后发生慢性粒细胞白血病一例

患者 ,女 ,5 7岁。因高热及面色苍白 1周 ,于 1997年 3月入院。入院前 3d在广州某医学院附属医院行骨髓检查 ,确诊为急性粒 单核细胞白血病 (AML M4b)。入院后查体 :体温 38.6℃ ,贫血貌 ,胸骨压痛 ,心、肺正常 ,肝肋缘下 2cm ,脾肋缘下 10cm。血常规 :Hb 6 5g/L ,WBC 80× 10 9/L ,BPC4 0× 10 9/L ,分类 :原始 早幼粒细胞 0 .4 6 ,嗜酸粒细胞0 .0 1,未见嗜碱粒细胞增多。骨髓检查 :有核细胞增生明显活跃 ,原始 早幼粒细胞 0 .2 70 ,原始 幼稚单核细胞 0 .38,嗜酸粒细胞 0 .0 2 0 ,嗜碱粒细胞占 0 .0 15 ,全片未见巨核细胞。POX…     

慢性粒-单核细胞白血病患者临床特征及生存分析

目的：探讨慢性粒-单核细胞白血病(CMML)患者的临床特征、预后因素和去甲基化药物(HMA)的疗效。方法：对37例初诊CMML患者的临床资料进行回顾性分析，总结其临床特征和HMA的疗效，应用Kaplan-Meier法和Log-rank检验进行单因素生存分析，Cox比例风险回归模型进行多因素分析。结果：患者中位发病年龄为67岁，常见症状包括乏力、出血、血象异常、发热等，多数患者存在脾大。根据FAB分型，发育异常型CMML 6例，增殖型CMML 31例；根据WHO分型，CMML-0 8例，CMML-1 9例，CMML-2 20例。诊断时中位白细胞计数32.84×10⁹/L，中位血红蛋白浓度(Hb)101 g/L，中位血小板计数65×10⁹/L，中位单核细胞绝对值9.53×10⁹/L，中位中性粒细胞绝对值(ANC)11.29×10⁹/L，中位乳酸脱氢酶(LDH)水平374 U/L。31例进行染色体核型分析或荧光原位杂交检测的患者中4例存在细胞遗传学异常，可分析结果的12例患者中11例存在基因突变，常见的有...     

地西他滨增强allo-NK细胞杀伤白血病干细胞敏感性研究

目的:探讨地西他滨增强allo-NK细胞杀伤白血病干细胞(LSC)的作用及其机制。方法:应用免疫磁珠法从KG1a细胞中分选LSC。从健康供者的外周血分离纯化同种异体反应性自然杀伤(allo-reactive natural killer,a1-Io-NK)细胞;LDH法检测allo-NK细胞对LSC的杀伤作用,流式细胞术检测allo-NK细胞诱导LSC凋亡及LSC表面NKG2D配体MICA/B和ULBP1-3的表达。结果:地西他滨10μmol/L处理LSC 24 h后,allo-NK细胞对LSC的杀伤率明显增高,在效靶比为5:1、10:1、20:1时分别为(60.52%±3.52%vs 22.08%±2.07%、73.93%±2.33%vs 28.99%±3.13%、83.08%±1.32%vs 36.44%±2.40%),差异有统计学意义(P0.05);地西他滨10μmol/L处理LSC 24 h后,在效靶比为10:1时,allo-NK细胞诱导LSC的凋亡率为7.84%±0.34%,明显高于LSC未处理组(3.33%±0.64%),差异有统计学意义(P0.05);地西他滨10μmol/L处理LSC 24 h后,LSC表面NKG2D配体(MICA/B、ULBP1、ULBP2、ULBP3)的表达上调,高于未处理组,差异有统计学意义(P0.05)。结论:地西他滨可能通过上调NKG2D配体增强allo-NK细胞对LSC的杀伤作用。     

老年人直肠癌合并急性粒-单核细胞白血病

1 病例资料 男,79岁,因排便不尽感、便次增多3个月入院.患者有高血压病史30年,糖尿病病史3年,长期口服药物治疗,入院时血压、血糖控制较好.平素无发热、消瘦,家族中无肿瘤患者.查体:血压140/90 mmHg.全身浅表淋巴结未触及明显增大,未见皮肤黏膜出血点.     

Red blood cell alloimmunization in transfused patients with myelodysplastic syndrome or chronic myelomonocytic leukemia

BACKGROUND: Red blood cell (RBC) alloimmunization is a major problem in chronically transfused patients because of the risk of hemolytic reactions and limited availability of compatible blood. This study was aimed at determining the characteristics of RBC alloimmunization in transfusion‐dependent patients with myelodysplastic syndrome or chronic myelomonocytic leukemia (MDS/CMML). STUDY DESIGN AND METHODS: The transfusion and clinical records of all patients with MDS/CMML seen at our hospital from 1990 to 2009 were reviewed. The cumulative incidence of RBC alloimmunization was calculated by taking death as a competing risk. Incidence rates were compared by Poisson multivariate regression. RESULTS: A total of 272 patients were included. Median age was 74 years; 55% were men and had received a median of 33 (range, 4‐421) RBC units. Forty‐two (15%) patients formed 81 alloantibodies and seven autoantibodies. Three additional patients developed autoantibodies without alloantibodies. The incidence rate of RBC alloimmunization was 1 per 10.5 person‐years and was independent of sex, age, and MDS diagnostic category. The cumulative incidence of alloimmunization increased with the number of RBC transfusions, reaching a plateau at 19.5% after 130 RBC units. The most common antibody specificities were Kell (26 cases), E (19), c (5), and Jk^a (5). In 26 (62%) of the 42 alloimmunized patients, only the Rh system and Kell were involved. CONCLUSION: RBC alloimmunization occurs in 15% of MDS/CMML patients on chronic transfusion support and mostly involves the Rh system and Kell. Transfusing these patients with extended antigen‐matched blood, including Kell and CcEe antigens, would presumably reduce the RBC immunization rate.     

Unusual skin lesions in chronic myelomonocytic leukemia

Chronic myelomonocytic leukemia (CMML) is a relatively rare, heterogeneous syndrome classified as a myelodysplastic syndrome according to the French-American-British classification system. The patient's presenting symptom was a pigmented skin nodule that, although common for cases of acute monoblastic leukemia, is peculiar for CMML. This case should increase awareness of the inclusion of CMML in the differential diagnosis of a discolored nodule and highlight the clinicopathologic considerations and therapeutic challenges consistent with the diagnosis of CMML.     

Monocyte subsets to differentiate chronic myelomonocytic leukemia from reactive monocytosis

BackgroundChronic myelomonocytic leukemia (CMML) is characterized by persistent monocytosis and dysplastic features of blood cells. No specific genetic abnormalities are present in CMML, and reactive monocytosis should be excluded. An increase in classical monocytes (MO1) has been suggested as a screening tool for CMML.MethodsWe evaluated monocyte subsets in the peripheral blood of patients with CMML (n = 16), patients with reactive monocytosis (n = 19), and normal controls (n = 15) with flow cytometry using antibodies against CD14, CD16, CD56, CD24, CD45, and CD2. The cutoff of MO1 ≥94% was validated, and the optimal cutoff was analyzed with receiver operating curve analysis.ResultsThe sensitivity of monocyte subset testing for screening for CMML was 0.938 (0.717‐0.997), and the specificity was 0.882 (0.734 ‐ 0.953) using the cutoff of MO1 ≥94%. Serial samples from patients who responded to hypomethylating therapy showed an MO1 < 94%. However, few patients with reactive monocytosis, including patients with nonhematologic malignancies and acute myeloid leukemia, showed an increase in the MO1 ≥ 94%. Monocyte subset results were correlated with the response to hypomethylating therapy in follow‐up samples.ConclusionMonocyte subset analysis is useful in screening for and monitoring CMML. Harmonization of the protocols for monocyte subset analysis is required.     

Interleukin 4 suppresses the spontaneous growth of chronic myelomonocytic leukemia cells.

We studied the effects of IL-4 on the spontaneous proliferation of chronic myelomonocytic leukemia (CMMoL) cells in vitro. IL-4 (100 U/ml) suppressed the spontaneous DNA synthesis by approximately 50% in 5 of 8 cases examined. IL-4 (100 U/ml) also inhibited the spontaneous colony formation by CMMoL cells in a methylcellulose culture by 50-97% in all of the 10 cases in which spontaneous colonies were formed. This IL-4-mediated suppression of the growth of CMMoL cells was completely abolished by the addition of anti-IL-4 neutralizing antibodies. The spontaneous CMMoL colonies were substantially suppressed by the addition of either anti-IL-6 or anti-granulocyte/macrophage colony-stimulating factor (GM-CSF) antibodies to the colony assay system: the addition of both anti-IL-6 and anti-GM-CSF antibodies resulted in greater than 80% inhibition of the colony formation by CMMoL cells. On the other hand, none of anti-IL-1-beta, anti-granulocyte-CSF, anti-macrophage-CSF, or anti-tumor necrosis factor-alpha antibodies affected the CMMoL colony formation. In the supernatants from 24-h cultures of CMMoL cells, high levels of IL-6 and GM-CSF were demonstrated in 9 of 9 and 2 of 9 cases examined, respectively. IL-4 (100 U/ml) almost completely inhibited the secretion of IL-6 and GM-CSF by CMMoL cells. These observations suggest that IL-4 suppresses the spontaneous proliferation of CMMoL cells by inhibiting their production of IL-6 and/or GM-CSF, both of which could act in vitro as an autocrine growth factor for CMMoL cells.     

格列卫治疗54例Ph阳性慢性髓细胞白血病

目的评价甲磺酸伊马替尼(商品名格列卫)治疗Ph阳性慢性髓细胞白血病慢性期(CML-CP)的有效性及安全性。方法54例对α干扰素(IFN-α)耐药或不能耐受及异基因干细胞移植(allo—SCT)后复发的CMI-CP患口服格列卫400mg／d，持续6-11个月。结果全部患于服药后7～28天达到血液学完全缓解。1例服药后7个月复发，很快进展为加速期。观察截止时，血液学完全缓解为52例(98％)。发生主要遗传学效应为37例(70％)，其中遗传学完全缓解为27例(51％)。37例中29例(78％)患达到主要遗传学缓解的时间为3个月。约10％患出现Ⅲ级白细胞和(或)血小板减少，多可控制或耐受。Ⅲ～Ⅳ级非血液学不良反应很少发生。仅1例患因药物不良反应退出。结论①格列卫对经IFN—α治疗失败的CMI-CP有较高的血液学及遗传学缓解率，且起效迅速；②格列卫服用方便，不良反应轻微、多可耐受或自行消失。     

A novel mutation of SETBP1 in atypical chronic myeloid leukemia transformed from acute myelomonocytic leukemia

To investigate an oncogenic mutation of SETBP1 in the evolution from acute myelomonocytic leukemia (M4) to secondary aCML. Clinical data and molecular studies were analyzed of paired aCML and 'normal'DNA from a case with M4. We identified a mutation in SETBP1 (encoding a p.Asp868Ala alteration). The analysis of paired sample indicated that SETBP1 mutation was acquired during leukemic evolution.     

Acute myelomonocytic and monoblastic leukemia with polyradicular symptoms

Meningeal leukaemia, developed in 4 female patients with M4 or M5 leukaemia during a period of haematological remission. Polyradicular symptoms and signs dominated neurologically, but 3 patients also exhibited cranial nerve palsies. The neurological findings showed no reversal following intrathecal chemotherapy with normalization of liquor cytology. Patchy demyelinization in the region of the anterior spinal roots and of the proximal segments of the affected cranial nerves were responsible for the neurological features. Peripherally located streaky demyelinization of the olfactory bulb and of the optic chiasm were not found to cause any neurological manifestations.