老年慢性粒单核细胞白血病的临床分析

目的探讨老年慢性粒单核细胞白血病(CMML)患者临床和血液学特点及相应治疗对策。方法分析该院确诊为CMML老年患者的资料。结果 CMML多见于老年人,有病态造血,治疗主要包括对症支持、化疗等方法,但治疗效果不理想,2年生存率低。结论老年CMML预后较差,在治疗中应充分考虑到疾病的特点、患者的机体状况等,寻找适合患者的最佳治疗方案以改善患者临床症状,提高生存质量。     

慢性粒单核细胞白血病合并多发性骨髓瘤1例

本文报道了1例慢性粒单核细胞白血病（CMML）与多发性骨髓瘤（MM）同时诊断的罕见病例。文献中仅有7例报道,均以MM治疗为主,本例为首例针对CMML采用阿扎胞苷去甲基化治疗,4程化疗后贫血好转、骨髓原始幼稚单核细胞消失、异常核型下降,而M蛋白和骨髓瘤细胞仍存在。讨论了两者共同发生的可能机制及诊治策略。     

老年慢性粒单核细胞白血病21例分析

慢性粒单核细胞白血病（ chronic myelomonocytic leukemia,CMML）在WHO分类中被归类到骨髓增生异常（ myelodysplastic syndromes , MDS ）/骨髓增殖性肿瘤（myeloproliferative neoplasms,MPN）[1],其表现既有骨髓病态造血的特征,同时又有骨髓增殖的特征。造血干细胞移植（hematopoietic stem cell transplantation,HSCT）是根治CMML唯一的方法,但对老年患者而言,HSCT并不是常规使用的治疗方法,其他对CMML有效的治疗并不多。对老年CMML患者来说,如何选择治疗方式和治疗时机对能否延长生存和提高生活质量是非常重要的问题。     

慢性粒单核细胞白血病治疗进展

慢性粒单核细胞白血病(chronic myelomonocytic leukemia,CMML)是一类具有骨髓增生异常综合征(myelodysplastic syndromes,MDS)和骨髓增殖性肿瘤(myeloid proliferative neoplasm,MPN)特征的恶性造血干细胞性疾病,是最具侵袭性的慢性...     

慢性单核细胞白血病一例

患者,女,60岁.因头晕、乏力一年余,加重伴皮肤黏膜出血点、黑便一月,于2004年2月入院.患者于一年前出现面色苍白、乏力,但未予重视,于一月前全身皮肤出现散在出血点及瘀斑,伴黑便,头晕、乏力症状加重,卧床不起,血常规示白细胞明显增高,于外院行骨穿提示为白血病,遂住入我科.查体：重度贫血貌;全身皮肤可见散在出血点及瘀斑;双侧颈部及左侧锁骨上均可触及数个黄豆大小淋巴结,活动度差;牙龈无肿胀;胸骨无压痛.     

慢性粒单核细胞白血病预后系统的循证评价

慢性粒单核细胞白血病(chronic myelomonocytic leukemia,CMML)是一类具有骨髓增生异常综合征(myelodysplastic syndromes,MDS)和骨髓增殖性肿瘤(myeloid proliferative neoplasm,MPN)特征的恶性造血干细胞疾病,是最具侵袭性的慢性白...     

慢性粒单核细胞白血病的临床特点和预后分析

目的:本研究探讨慢性粒单核细胞型白血病(CMML)的临床特点及影响其预后的相关因素,评估IPSS、WPSS及MDAPS3个积分系统在CMML预后中的应用意义。方法:选取我院2003年5月-2009年7月CMML病例18例,选取同期急性单核细胞白血病(AML-M5)58例作为对照。结果:CMML伴有明显的多系病态造血。干细胞相关抗原CD34、CD117的表达较低,HLA-DR、CD117、CD14抗原表达均低于AML-M5(P<0.05),CD11b抗原表达高于AML-M5(P<0.05)。结论:WPSS积分系统对CMML的预后评估较为理想;CMML时干细胞相关抗原的表达均较低;CD11b高表达可能与CMML的预后不良有关;常规化疗对CMML的治疗效果不佳,造血干细胞移植是目前惟一有可能治愈CMML的方法。     

58例急性粒-单核细胞和急性单核细胞白血病的临床及治疗疗效的比较

本文总结了24例 M_4和34例 M_5患者的临床和治疗疗效观察。研究结果表明,M_4好发于女性而 M_5多见于男性,在>60岁老人中 M_5较 M_4多见.M_4患者出血和贫血较明显,M_5以高热、肝脾浸润多见,在治疗反应上,无论 HOAP 或其它化疗方案组,M_5的 CR 率低于 M_4患者。     

Chronic myelogenous leukaemia with p185(BCR/ABL) expression: characteristics and clinical significance

We investigated the significance of p185BCR/ABL expression in patients with chronic myelogenous leukaemia (CML) in relation to disease features, therapy and outcome. Results of Western blot analysis for 1384 patients referred with a diagnosis of CML to our institution from 1989 to 1997 were reviewed. Clinical characteristics, results of cytogenetic analysis and RT-PCR for BCR rearrangement were analysed. Five patients with Ph-positive CML expressing the p185BCR/ABL hybrid protein were identified. By RT-PCR, bone marrow specimens of these patients were confirmed to have an e1a2 junction. The median age at diagnosis of these patients was 55 years (range 43-76). All had elevated white cell counts at diagnosis (median 50 x 109/l, range 11.7-163 x 109/l). Four patients had monocytosis (range 10-16%) with a low neutrophil/monocyte ratio in the peripheral blood (range 3.4-5.7). Patients presented with various stages of the disease (two in chronic-phase CP, two in accelerated-phase AP, and one in blastic-phase BP). The clinical course and therapy of the patients varied, with one patient receiving hydroxyurea only, three patients receiving hydroxyurea followed by interferon-alpha based regimens and bone marrow transplantation. The patient presenting in BP was treated with combination chemotherapy. The clinical outcome of the patients was also varied with one patient alive and in complete remission (with complete cytogenetic remission after transplant) and four patients dead after progression to more advanced stages. We conclude that patients with Ph-positive p185BCR/ABL CML frequently present with monocytosis and a low neutrophil/monocyte ratio in the peripheral blood, aiding the speculation that the presence of the p185BCR/ABL hybrid protein may contribute to a phenotype intermediate between CML and CMML. Of interest, the only other specific clinical feature identified was the absence of splenomegaly in four of five patients. There was no definite association with transformation to lymphoid blast phase.     

影响慢性粒细胞白血病长期生存的因素临床分析

本文把存活5年以上的23例慢粒患者与同期生存时间少于5年的40例慢粒患者的临床特点进行了对照分析;发现生存期较长病例的脾肿大较轻,其首次缓解持续时间较长,而白细胞较高的患者,预后并不一定差。     

Imatinib Mesylate in Combination with Other Chemotherapeutic Agents for Chronic Myelogenous Leukemia

Imatinib therapy is an important contribution to the management of patients with chronic myelogenous leukemia (CML). Despite high rates of hematologic and cytogenetic responses to imatinib therapy, the emergence of resistance to imatinib has been recognized as a major problem in the treatment of CML. Experimental and clinical studies suggest that imatinib as a single drug may not be sufficient to eradicate BCR-ABL-positive stem cells. Therefore, whether combinations of imatinib with other agents can increase the length of molecular remission and whether such combinations can prolong survival should be determined by large-scale clinical studies. In this review, we discuss efficacious combinations for future clinical trials. These regimens combine imatinib with conventional chemotherapeutic agents or with inhibitors of other signal transduction molecules that may be preferentially activated in CML cells.     

Multiple Constitutional Chromosome Translocations of Familial Nature in Philadelphia Chromosome-Positive Chronic Myeloid Leukemia: A Report on a Unique Case

A case of Philadelphia chromosome-positive (Ph(+)) chronic myeloid leukemia (CML) featuring 2 additional balanced translocations, t(2;5) and t(6;12), as well as a robertsonian translocation, t(13;14), was diagnosed by routine bone marrow karyotyping. The breakpoints did not involve previously described CML-related chromosomal regions in any of the 3 translocations. Despite the patient's partial response following imatinib therapy, all Ph(-) bone marrow metaphases persistently had the 3 additional chromosomal changes. Moreover, stimulated peripheral B-lymphocytes from the patient also showed the same chromosomal changes, suggesting that we had found a complex constitutional chromosome aberration unrelated to the leukemia. Peripheral blood karyotype analyses of 6 of the 7 closest relatives from 3 generations demonstrated at least 1 of these aberrations, although in different combinations. Standard bone marrow or peripheral blood karyotyping of hematologic disorders may uncover otherwise symptomless, unrelated constitutional changes together with disease-specific chromosome aberrations.A triple constitutional chromosome aberration combined with a hematologic disorder has not been described until now. In addition, multiple constitutional aberrations persisting through at least 3 generations seem to be extremely rare. At present, no direct evidence exists to support a causative relationship between the familial translocations and leukemogenesis.     

Chronic myelomonocytic leukemia: a test of a proposed staging system

In an attempt to characterize the prognostic significance of the main clinical and hematological features of chronic myelomonocytic leukemia (CMML), 30 such patients were allocated the "modified Bournemouth score". The most significant separation of groups predictive of poorer survival was obtained by setting a cutoff at the median score value for the whole series (score,2). On this basis patients could be divided into two different groups whose survival probability as well as risk of acute transformation significantly differ each other (P less than 0.05). In our opinion, this score could be of interest in treatment planning of CMML patients when the possibility of conservative or aggressive approach in envisaged.     

Use of Imatinib Mesylate for Favorable Control of Hypercalcemia Mediated by Parathyroid Hormone-Related Protein in a Patient with Chronic Myelogenous Leukemia at Blast Phase

The case of a 72-year-old woman with chronic myelogenous leukemia in blast phase (BP) with hypercalcemia is reported. Bone x-ray examination revealed multiple osteolytic lesions throughout the body. The serum level of parathyroid hormone-related protein (PTHrP) was elevated, and PTHrP messenger RNA (mRNA) was detectable in the peripheral blood mononuclear cells (PBMNC) at BP but was not detectable at chronic phase (CP).Treatment with conventional chemotherapy did not completely control either serum calcium level or serum PTHrP level. Treatment with imatinib mesylate (imatinib) alone rapidly normalized these parameters in parallel with a decrease in the number of blast cells. The treatment also maintained the patient in good condition for approximately 3 months, even though the number of blast cells, serum calcium level, serum PTHrP level, and PTHrP mRNA level increased at the terminal stage. Mutations of the p53, K-Ras, and BCR-ABL genes in PBMNC at BP were absent. A noteworthy feature in this patient was that PBMNC at BP but not at CP showed high Lyn mRNA expression. Taken together the findings showed that production of PTHrP by blast cells was favorably controlled by imatinib therapy alone. Imatinib may prolong survival time at BP even though the patients have the complication of PTHrP-mediated hypercalcemia.     

Total Leukocyte Counts and the Requirement of Dose Reduction due to Cytopenias as Prognostic Indicators Affecting Response to Imatinib in Chronic Myeloid Leukemia

Imatinib is a tyrosine kinase inhibitor and is considered the first line of non stem cell transplantation treatment for patients diagnosed with CML. We evaluated the response rates and adverse reactions to Imatinib in our patients and tried to identify factors which affected the response to Imatinib. Eighty-four patients were diagnosed on the basis of clinical and haematological variables with confirmation by FISH, detecting Philadelphia chromosome or bcr-abl translocation and were then started on oral capsule Imatinib. A complete haematological response was seen in 78.04% patients, while complete cytogenetic response (CCR) was seen in 12.2% of patients and major cytogenetic response (MCR) was seen in 64.63% of patients. It was found that that a greater total leukocyte count (TLC) on presentation had a negative correlation with cytogenetic response. Cytopenias were seen in 36 patients (43.82%). 34.9% of patients having CCR/MCR required dose reduction while 73.6% of patients not achieving CCR/MCR required dose reduction. This was a significant difference, confirmed on statistical analysis (P < 0.05; P = 0.019), establishing the negative prognostic value of dose reduction due to cytopenias.