华氏巨球蛋白血症继发急性髓系白血病一例并文献复习

目的:提高对华氏巨球蛋白血症（WM）继发急性髓系白血病（AML）的认识。方法:回顾性分析江苏省盐城市第一人民医院2018年2月收治的1例WM治疗后继发伴复杂染色体异常的AML患者的临床资料，并结合文献探讨WM继发AML的发病机制和预后。结果:该患者为老年男性，初诊为WM，COP、氟达拉滨单药等方案化疗后106个月诊断为AML，染色体核型分析示5号和7号染色体均异常，并伴其他染色体异常的复杂核型。患者拒绝化疗、后死于肺部感染，生存期不足1个月。结论:WM继发AML少见，目前发病机制尚不明确，其发生可能与应用烷化剂、核苷类似物、遗传易感性及谱系转换等因素有关，伴复杂染色体异常的继发性AML患者生存期较短，预后差。     

伴rob(13;22)(q10;q10)的治疗相关急性髓系白血病1例并文献复习

目的提高对治疗相关急性髓系白血病(AML)的认识。方法回顾性分析烟台毓璜顶医院收治的1例横纹肌肉瘤治疗后继发AML伴染色体核型rob(13;22)(q10;q10)患儿的临床资料,并进行文献复习。结果该例女性患儿6岁5个月,在2018年1月诊断为横纹肌肉瘤,接受规范化疗30个月后确诊为治疗相关AML,染色体核型45,XX,t(11;19)(q23;q13),rob(13;22)(q10;q10),融合基因MLL⁃ELL阳性。接受DA(阿糖胞苷+柔红霉素)方案化疗后无明显改善,家属放弃治疗,患儿死亡。结论治疗相关AML多与应用鬼臼毒素类(依托泊苷等)药物化疗、联合放疗等因素有关。具有高危细胞遗传学因素的治疗相关AML预后差。     

t（8;21）（q22;q22）急性髓系白血病转化为急性淋巴细胞白血病一例并文献复习

目的 探讨t（8; 21） （q22; q22）急性髓系白血病（AML）转化为急性淋巴细胞白血病（ALL）的克隆转化机制.方法 报道1例初诊为t（8; 21）AML,后转化为ALL患者的临床资料.期间对其进行持续细胞形态学、遗传学和分子生物学监测.结果 患者人院时诊断为t（8;21）AML,AML1-ETO融合基因阳性,治疗后达到完全缓解.半年后转化为ALL,诱导化疗后再次缓解.结论 t（8;21）（q22;q22）AML转化的ALL可能起源于具有髓/淋分化潜能的多能造血干细胞,早期化疗抑制了占优势地位的髓系白血病克隆,从而使具有不同表型的淋系亚克隆增殖.     

CAG方案与普通化疗方案治疗老年人急性髓系白血病的比较观察

目的探讨CAG方案治疗老年人急性髓系白血病（AML）疗效及不良反应。方法共选择老年AML患者45例,治疗组23例应用CAG方案,对照组22例应用DA、HA或MA方案。结果治疗组1个疗程完全缓解率（CR）率39．1％,有效率57．7％;对照组CR率31．8％,有效率54．5％,两组间差异无统计学意义（P〉0．05）。两组间骨髓恢复时间、所需浓缩红细胞及单采血小板数,差异有统计学意义（P〈0．05）。结论CAG治疗老年人AML疗效肯定,较普通化疗方案骨髓抑制时间短,输血量减少。     

伴有6号染色体长臂缺失的急性髓细胞白血病二例报告及文献复习

 目的　了解6q-异常急性髓细胞白血病（AML）的临床和生物学特点。方法　报道2例伴有6号染色体长臂缺失(6q-)的AML,并对伴有6q-异常AML的有关文献进行复习。结果　2例患者分别诊断为AML-M1和AML-M2,均表达髓系抗原,不表达淋巴细胞抗原。染色体核型分别为：46,XX, del(6)(q21q25),t(4;7)(q10;q10)[3]/46,XX,del(6)(q21q25)[2]/46,XX[25]以及46,XX,del(6)(q23),t(7;11)(p15;p15)[5]/46,XX,t(7;11)(p15;p15)[9]/46,XX[6]。现有文献共报道了伴有6q-异常AML 28例（包括该组报道的2例）。大部分患者伴有附加染色体异常。6q-的断裂点广泛分布于q12-q27,但主要累及6q21-q23区域。总体看来,伴有6q-异常的AML对化疗效果差、生存期短。6q-异常克隆本身可能导致了AML的临床恶性过程。AML患者出现6q-,可能与6号染色体长臂上myb以外癌基因的激活或抗癌基因的丢失有关。结论　AML伴有6q-异常很少见,具有自身的生物学特点,临床预后不良。     

维奈克拉联合阿扎胞苷治疗初诊复杂染色体核型老年人急性髓系白血病一例并文献复习

目的:提高对维奈克拉联合阿扎胞苷治疗复杂染色体核型的老年人急性髓系白血病的认识。方法:回顾性分析2020年7月解放军联勤保障部队第九四〇医院收治的1例复杂染色体核型老年急性髓系白血病患者的临床资料，并进行相关文献复习。结果:该例患者诊断为急性髓系白血病M 2，WT1基因阳性，高危组，染色体核型为50-51，XX，add（3）（q12），+6，add（11）（q25），-？15，+21，+22，+mar1，+mar2，inc[cp8]。入院后给予2次维奈克拉联合阿扎胞苷方案治疗，治疗过程未发生不良反应，耐受性良好，达到完全缓解，患者出院后失访。 结论:以复杂染色体核型为特征的老年人急性髓系白血病诊治难度较大，临床异质性大，以维奈克拉为代表的bcl-2抑制剂治疗老年人急性髓系白血病的化疗方案短期内不良反应小，相较常规化疗方案缓解率高，长期疗效尚需进一步观察。     

循环应用CAG和IA方案治疗老年急性髓性白血病的临床观察

目的:观察循环应用CAG、IA方案治疗老年初治急性髓性白血病(AML)的疗效及不良反应.方法:25例老年初治AML患者分为CAG、IA循环化疗组(Ⅰ组)以及常规方案化疗组(Ⅱ组),Ⅰ组10例患者给予CAG方案治疗,如达不到完全缓解原方案再次治疗,完全缓解后给予IA方案化疗.间歇1个月后再循环应用CAG、IA方案化疗;Ⅱ组15例患者予常规柔红霉素(DNR)+阿糖胞苷(DA)或米托蒽醌+阿糖胞苷(MA)方案化疗,完全缓解后应用DA、MA、HA和IA等方案化疗.结果:Ⅰ组2个周期完全缓解率为60%(6/10),总有效率达到80%(8/10),而Ⅱ组2个周期的完全缓解率仅为26.7%(4/15),总有效率为53.3%(8/15);Ⅰ组无化疗相关死亡病例,Ⅱ组化疗相关死亡率为20%.结论:循环应用CAG、IA方案治疗老年初治AML患者较传统常规化疗方案治疗具有完全缓解率及2年生存率高,毒副反应小的优点.     

老年急性髓细胞白血病的疗效观察

 目的 研究老年急性髓细胞白血病（AML）的临床特点,寻求治疗的有效策略。方法 回顾性分析30例老年AML,在积极支持治疗下,按个体差异采取不同的化疗方案进行化疗。结果 小剂量阿糖胞苷（LD-Ara-C）组治疗7例,完全缓解（CR）率14.3 %;MA组治疗6例,CR率50.0 %;DA组治疗7例,CR率42.8 %;CAG组治疗10例,CR率60.0 %。结论 老年AML对化疗反应差,CR率低,治疗应选择积极合理、个体化的化疗方案。CAG方案CR率高,毒副作用小,治疗老年AML有一定的优势。     

129例成年人急性白血病染色体核型分析

 目的 探讨急性白血病（AL）的细胞遗传学改变，了解染色体核型异常与AL FAB分型的关系及其对预后因素的影响。方法 129例AL患者于治疗前抽取骨髓标本，采用短期细胞培养法制备染色体标本，应用吉姆萨显带技术进行染色体核型分析。结果 进行染色体检查的129例AL患者中，21例无分裂象，在108例可供染色体核型分析的患者中，染色体核型异常65例（60.2 %）；在急性淋巴细胞白血病（ALL）、急性髓细胞白血病（AML）和急性混合细胞白血病（AMLL）3种类型的白血病患者中，染色体核型异常分别为54.2 %（13／24）、63.2 %（48／76）和50.0 %（4／8）；在染色体核型异常的65例患者中，超二倍体9例（13.8 %），亚二倍体12例（18.5 %），假二倍体2例（3.1 %），正常二倍体42例（64.6 %）。与FAB分型相关的特异性染色体重排35例（AML 26例、ALL 8例、AMLL 1例），占53.8 %，而在AML、ALL和AMLL染色体核型异常的患者中，与FAB分型相关的特异性染色体重排分别为54.2 %（26／48）、61.5 %（8／13）和25.0 %（1／4）。结论 60 %的AL存在克隆性染色体异常，一些特异性染色体异常改变是AL的细胞遗传学特征，与AL的FAB分型有明显相关性，不仅是诊断AL的重要依据，也是AL化疗和造血干细胞移植治疗后疗效观察、微小残留病（MRD）监测及判断预后和早期预测复发的重要指标。     

CAG方案治疗老年人急性髓系白血病20例

 【摘要】 目的 观察CAG方案治疗老年人急性髓系白血病（AML）的疗效及安全性。方法 回顾性分析接受CAG方案治疗的老年AML患者20例的临床资料。CAG方案为阿糖胞苷10 mg／m2,每12 h皮下注射,第1天至第14天;阿柔比星每天10～14 mg／m2,静脉注射,第1天至第4天;粒细胞集落刺激因子每天200 μg／m2,皮下注射,第1天至第14天。14 d为1个疗程,1～2个疗程结束评价疗效。治疗1个疗程达完全缓解（CR）者用常规DA、HA、MA方案巩固治疗,连用2个疗程不缓解者则更换方案。观察治疗效果及患者不良反应。结果 20例患者中18例完成治疗,其中CR 5例,部分缓解（PR）7例,CR率27.8 ％（5／18）,总有效率66.7 ％（12／18）。除骨髓抑制外,无严重感染、出血及重要脏器损伤。结论 CAG方案治疗老年人AML疗效确切,相对安全,耐受性好。     

Prevention of chemotherapy-induced leukemia and of leukemia relapses

Fifty percent of patients with the myelodysplastic syndrome, frequently following treatment by radiation or chemotherapy, have prognostically unfavorable deletions of the long arms of chromosomes 5 and 7, or trisomy 8, as have the 25% of patients with acute myeloblastic leukemia where remissions last 6–12 months, and where relapse cannot be prevented. In contrast, patients with prognostically favorable cytogenetics (translocation 15; 17 or 8; 21 or inversion 16) maintenance chemotherapy may prevent relapses. Of chronic myelocytic leukemia patients, 85% can achieve hematological remission with interferon α, and 40% a partial cytogenetic remission, which probably delays relapse.     

Meningeal leukemia complicating chronic lymphocytic leukemia

Two patients with classic chronic lymphocytic leukemia had meningeal leukemia as a complication of their disease. Intrathecal chemotherapy was successful in eradicating signs and symptoms of meningeal involvement. One of these patients is alive without evidence of central nervous system leukemia 30 months after diagnosis of meningeal leukemia, and 5 1/2 years after the diagnosis of chronic lymphocytic leukemia. Although uncommon, meningeal involvement in chronic lymphocytic leukemia may occur at various times in the course of the disease, it responds to conventional therapy.     

Prevention of chemotherapy-induced leukemia and of leukemia relapses.

Fifty percent of patients with the myelodysplastic syndrome, frequently following treatment by radiation or chemotherapy, have prognostically unfavorable deletions of the long arms of chromosomes 5 and 7, or trisomy 8, as have the 25% of patients with acute myeloblastic leukemia where remissions last 6-12 months, and where relapse cannot be prevented. In contrast, patients with prognostically favorable cytogenetics (translocation 15; 17 or 8; 21 or inversion 16) maintenance chemotherapy may prevent relapses. Of chronic myelocytic leukemia patients, 85% can achieve hematological remission with interferon alpha, and 40% a partial cytogenetic remission, which probably delays relapse.     

Association of B-chronic lymphocytic leukemia and acute myeloid leukemia

A 62-year-old man presented with fatigue, pallor and mild weight loss. Laboratory studies showed Hb 7.6 g/dl, Hct 21.8%, WBC 108x10(9)/1, PLT 143x10(9)/1. At morphological examination, circulating cells appeared as 60% blasts and 40% lymphocytes, with smudge cells. A bone marrow aspirate showed infiltration by blasts (50%) and lymphocytes (40%); alpha-naphtyl-acetate esterase was positive in 90% of blasts, while myeloperoxidase was positive in 10%. The immunologic phenotype of blasts was characterized by the co-expression of CD13, CD33, CD14, CD4, CD15, CD64, CD117, HLA-DR, CD11b. Lymphocytes were characterized by a B-CLL immunophenotype: CD19+, CD5+, CD23+, CD20+(dim), FMC7+(dim), K light chain+(dim). Karyotype was normal and PCR assays for AML-ETO, CBFbeta-MYH11, PML-RARalpha, BCR-ABL and bcl-1/JH translocation were negative. Coexistence of CLL and AML with monoblastic features was diagnosed. Simultaneous appearance of CLL and AML has rarely been described and represents a peculiar biological phenomenon.     

Detection of minimal residual leukemia in acute lymphoblastic leukemia

Morphologic, immunologic, enzymatic and cytogenetic methods detect residual leukemic cells at the level of 5%. The early detection of leukemia by these methods has failed to improve outcome for children with residual disease. The use of molecular biologic techniques, particularly those involving the polymerase chain reaction, can detect 1 leukemic cell among 10,000 or even 100,000 normal cells. At this time, it is essential to conduct the proper clinical trials to determine the clinical utility of methods so sensitive and specific.     

Differentiation of Abelson murine leukemia virus-infected promonocytic leukemia cells

Virus-producing, tumorigenic, promonocytic leukemia cell lines were derived from Abelson murine leukemia virus-infected mice. This study shows that, of these 25 cloned lines, 22 were capable of extensive differentiation. It also shows that granulocyte-macrophage colony-stimulating activity increased the proportion of differentiating cells in 17/22 lines. Cells from agar colonies with a diffuse colony morphology had an increased expression of mature macrophage phenotypic characteristics, and a reduced proliferative capacity in vitro, compared to cells from agar colonies with a compact colony morphology. Cells from diffuse colonies also produced less Abelson virus, and were less tumorigenic in vivo than cells from compact colonies. Together, these results suggest some Abelson virus-producing leukemic cells are not blocked in their capacity to differentiate and are capable of reversing the transformed phenotype.     

Coexistence of chronic myelogenous leukemia and chronic lymphocytic leukemia

A 55-year-old man is reported who initially developed chronic lymphocytic leukemia. Seven years later, after chemotherapy with chlorambucil, chronic myelogenous leukemia was diagnosed in addition to the chronic lymphocytic leukemia. Four previously reported cases with the same sequence of events are reviewed as well as cases of chronic myelogenous leukemia following chemotherapy alone.     

Dynamics of lymphocytic subpopulations in Friend leukemia virus-induced leukemia

The in vivo roles of the immunosurveillance mechanism of the host against leukemia induced by Friend leukemia virus (FLV) were examined. The significance of T-cells in host defense against FLV-induced leukemia was indicated by the fact that thymus-deprived C57BL/6N-nu/nu mice were sensitive to FLV, although normal C57BL/6N mice were, as already reported by many authors, resistant to FLV. In relation to the role of T-cells on the onset of FLV-induced leukemia, the population dynamics of the lymphocytic subpopulations of the systemic lymphoid organs after FLV injection in FLV-resistant C57BL/6N mice were examined in comparison with the dynamics in FLV-sensitive strains, C57BL/6N-nu/nu mice and normal C3H/HeN mice. In this system, Lyt-1+2- helper T-cells in the spleen of FLV-resistant C57BL/6N mice increased in number after FLV injection. The number of immunoglobulin positive cells did not remarkably change in FLV-resistant C57BL/6N mice after FLV injection, whereas the number increased in the lymph node of FLV-sensitive C3H/HeN mice. The results indicated that a major contribution to the relative susceptibility and resistance of the host to FLV was controlled by the capacity to mobilize T-cells to the spleen in an early stage of disease, although the interaction of these T-cells with other immune cells may play an important role in mediating host resistance to FLV-induced disease.     

Chronic lymphocytic leukemia and hairy-cell leukemia.

The chronic lymphoid leukemias are a heterogeneous group of disorders with different immunologic, biologic, and clinical features. The most common of these are the B-cell diseases, chronic lymphocytic leukemia and its variants, including prolymphocytic leukemia and hairy cell leukemia. The increased use of immunophenotyping has identified a number of other less common but related disorders. Despite being clonal disorders, the chronic B-cell leukemias exhibit immunologic abnormalities in multiple other lineages, the mechanism for which is not clear. Fludarabine, 2'-deoxycoformycin, and 2-chlorodeoxyadenosine are purine analogues that have advanced the treatment of chronic B-cell leukemias. Fludarabine appears to be the single most effective agent for chronic lymphocytic leukemia, while 2'-deoxycoformycin and 2-chlorodeoxyadenosine are both extremely effective in hairy cell leukemia. A recently completed comparison of alpha-interferon with 2'-deoxycoformycin in hairy cell leukemia may redefine the standard therapy for this disorder. Continued interaction between laboratory and clinical scientists is essential for continued progress in these diseases.