CD116在急性白血病中的表达及临床意义

目的：探讨ＣＤ１１６在急性白血病中的表达及临床意义。方法：对１１４例急性白血病者进行免疫表型及细胞遗传学分析。结果ＣＤ１１６在急性淋巴细胞白轿病（ＡＬＬ）中无阳性表达，而在急性髓性细胞白血病（ＡＭＬ）中阳性表达率为４２．４％；ＣＤ１１６在ＡＭＬ各亚型间出现的频率存在明显差异，阳性率Ｍ５为８３．３％，Ｍ４为４０．０％，Ｍ３和Ｍ２分别为２７．２％和１５．０。２例Ｍ５患者出现染色体＋８异常，伴ＣＤ１１６     

强烈化疗治疗小儿急性淋巴细胞白血病临床研究

目的：观察强烈化疗治疗小儿急性淋巴细胞白血病（ＡＬＬ）的疗效。方法：８３ 例ＡＬＬ患儿用ＣＯＤＰＬ方案诱导治疗,缓解后用ＣＡＴ、ＨＤＭＴＸ、ＥＡ、ＶＰＤＬ方案早期巩固强化治疗,用ＭＴＸ、６－ＭＰ、ＶＰ、ＣＯＰ、Ａｒａ－Ｃ维持治疗,用ＣＯＡＰ、ＥＡ、ＶＰＤＬ方案定期加强治疗,用ＨＤＭＴＸ联合三联鞘注对庇护所白血病进行预防。结果：完全缓解（ＣＲ）率９６．４％ （８０／８３）;３ 例在诱导期死于败血症。６８例坚持治疗的患儿,中位随访３７（１８～１０８）个月,５ 年持续完全缓解（ＣＣＲ）率７６．２％ 。标危ＡＬＬ和高危ＡＬＬ的５ 年ＣＣＲ率分别为７９．６％ 和７２．９％ 。结论：强烈化疗及坚持治疗是提高小儿ＡＬＬ５年ＣＣＲ率的关键;严重感染仍是治疗失败的主要原因。     

急性白血病免疫分型中的少见类型多系表达及"裸型"分析

目的：研究急性白血病（ＡＬ）细胞抗原表达特征及临床意义。方法：选用１４～１６ 种单克隆抗体,采用免疫荧光的方法,对９６例ＡＬ患者进行免疫分型。结果：９ 例为多系表达,即同时表达三系以上的抗原（急性髓细胞白血病－ＡＭＬ７ 例,急性淋巴细胞白血病－ＡＬＬ２ 例）,ＡＭＬ中多系表达发生率为１０．８％ ,ＡＬＬ中为６．５％ 。“裸细胞”型的发生率ＡＭＬ中７．７％ ,ＡＬＬ中６．４％ 。结论：呈多系表达的ＡＭＬ多为Ｍ５ 型,ＣＤ１４高表达,Ｐ－１７０ 高表达,ＣＲ率低;ＡＬＬ则发生于复发的病人     

老年人急性白血病细胞形态学、免疫学和细胞遗传学特征

目的 探讨初治老年人急性白血病（ＡＬ）免疫学和细胞遗传学特征。 方法 对４８例初治老年人ＡＬ采用间接免疫荧光技术进行免疫分型及Ｒ带或Ｇ带显带技术核型分析。 结果 形态学分型与形态学、免疫学、细胞遗传学（ＭＩＣ）分型４８例（９３．８％）相符合，３例（６．２％）为杂合型急性白血病。４８例老年人ＡＬ与５８例非老年人ＡＬ比较，急性髓细胞白血病（ＡＭＬ）发生率高，而急性淋巴细胞白血病（ＡＬＬ）发生率低（均为     

急性白血病合并DIC53例临床分析

对５３例ＡＬ合并ＤＩＣ作回顾分析，并发ＤＩＣ的ＡＬ类型为ＡＰＬ２８例，非Ｍ３型ＡＮＬＬ１５例、ＡＬＬ１０例，发生于初发期、诱导化疗和化疗间歇期、难治期的ＤＩＣ分别占２５、１１、１７例。结果显示，ＡＰＬ合并ＤＩＣ６８％发生于初发期、非Ｍ３期ＡＮＬＬ并ＤＩＣ发生于难治期占６０％，而ＡＬＬ并ＤＩＣ５０％与诱导化疗有关。４０例经全程治疗者，ＤＩＣ治愈８例，其中６例为初发期患者（６／１６）。讨论了ＤＩＣ发生     

急性早幼粒细胞白血病PML/RARα融合基因研究及其临床意义

目的 研究急性早幼粒细胞白血病（ＡＰＬ）ＰＭＬ／ＲＡＲα基因的亚型、变化以及与临床疗效、预后的关系。方法 采用筑巢式逆转录酶－聚合酶链反应（ＲＴ－ＰＣＲ）技术，检测初治ＡＰＬ患者ＰＭＬ／ＲＡＲα融合基因的ＲＮＡ异构体，监测完全缓解（ＣＲ）ＡＰＬ患者微小残留病（ＭＲＤ）。结果 ４９例且的ＡＰＬ患者ＰＭＬ／ＲＡＰα晤基因亚型，Ｓ型为４０．８％，Ｌ型较Ｓ型预后较好。分别采用三氧化二砷（ＡＳ２Ｏ３）、全反     

小剂量HA方案治疗急非淋白血病21例临床分析

本文报告了应用小剂量三尖杉酯碱－阿糖胞甘（ＬＤ－ＨＡ方案）治疗急非淋白血病（ＡＮ－ＬＬ）２１例，完全缓解（ＣＲ）１４例（６６．７％），部分缓解（ＰＲ）３例（１４．５％），总有效率８１．２％。认为ＬＤ－ＨＡ方案对ＡＮＬＬ效果满意，尤其是骨髓增生程度较低和血液中白细胞不增多的白血病效果较好。建议病人缓解后加强巩固治疗，以进一步延长生存期。     

自体造血干细胞移植治疗恶性血液病、肺癌临床观察

用自体造血干细胞移植（ＡＨＳＣＴ）治疗３例急性非淋巴细胞性白血病（ＡＮＬＬ）、１例霍奇金病（ＨＤ）和１例晚期肺癌（ＡＬＣ）。其中４例接受自体外周血干细胞移植（ＡＰＢＳＣＴ），１例接受自体骨髓移植（ＡＢＭＴ）。ＡＮＬＬ、ＨＤ、ＡＬＣ分别用ＭＡＣ、ＳＥＡＭ、ＳＥＥＣ方案预处理。５例患者移植后均获造血重建。ＡＮＬＬ和ＨＤ已分别持续完全缓解４８９天、３７２天、３３５天、２３０天；１例ＡＬＣ患者在移植后完全缓解１１５天后出现颅内复发。初步结果提示ＡＨＳＣＴ能延长ＡＮＬＬ、ＨＤ和ＡＬＣ患者的无病存活期     

淋巴系统恶性血液病患者血清IL-8的测定

目的；探讨血清ＩＬ－８变化在淋巴系统恶性血液病（ＬＭＨＤ）中的意义。方法：采用夹心酶联免疫吸附法检测３１例ＬＭＨＤ患者血清中ＩＬ－８水平。结果；急性淋巴细胞白血病９ＡＬＬ０与恶性淋巴瘤（ＭＬ）患者前ＩＬ－８水平显著高于正常对照组；非霍奇金淋巴瘤（ＮＨＬ）患者ＩＬ－８水平高于霍奇金病（ＨＤ）患者；ＬＭＨＤ患者治疗有效者ＩＬ－８水平显著下降，无效者仍保持高水平，完全缓解时与正常对照组无差异。     

小剂量化疗加维甲酸治疗急性白血病和骨髓增生异常综合征

小剂量化疗加维甲酸治疗急性白血病和骨髓增生异常综合征杨晓凤，陆岩，贺丹，宁辉我们自１９８９年８月用小剂量阿糖胞苷（ＬＤＡｒａ－ｃ）、小剂量三尖杉酯碱（ＬＤＨＡ）和全反式维甲酸（ＲＡ）联合治疗急性非淋巴细胞白血病（ＡＮＬ）和骨髓增生异常综合征（ＭＤＳ）...     

45例初治高白细胞性急性白血病的治疗效果与预后分析

目的:探讨初治高白细胞急性白血病(HAL)的临床特征、治疗效果及预后分析。方法:对45例初治HAL进行临床回顾性分析,同时以371例非高白细胞急性白血病(NHAL)作对照组。结果:HAL构成比为10.8%。HAL组的髓外浸润、DIC、白细胞淤滞综合征及早期死亡率较对照组高。HAL组完全缓解(CR)率为28.9%,低于NHAL的57.1%,早期病死率是17.8%。早期死亡的主要原因是颅内出血和呼吸窘迫综合征。HAL的缓解率和早期死亡率与FAB分型有关。结论:HAL完全缓解率低,早期死亡率高,预后差,特别是HAL-M3的早期死亡率高,要重视其早期处理。HAL的早期积极治疗可降低早期死亡率。     

Long term impact of hyperleukocytosis in newly diagnosed acute myeloid leukemia patients undergoing allogeneic stem cell transplantation: An analysis from the acute leukemia working party of the EBMT

Up to 20% of acute myeloid leukemia (AML) patients present initially with hyperleukocytosis, placing them at increased risk for early mortality during induction. Yet, it is unknown whether hyperleukocytosis still retains prognostic value for AML patients undergoing hematopoietic stem cell transplantation (HSCT). Furthermore, it is unknown whether hyperleukocytosis holds prognostic significance when modern molecular markers such as FLT3‐ITD and NPM1 are accounted for. To determine whether hyperleukocytosis is an independent prognostic factor influencing outcome in transplanted AML patients we performed a retrospective analysis using the registry of the acute leukemia working party of the European Society of Blood and Marrow Transplantation. A cohort of 357 patients with hyperleukocytosis (159 patients with white blood count [WBC] 50 K‐100 K, 198 patients with WBC ≥ 100 K) was compared to 918 patients without hyperleukocytosis. Patients with hyperleukocytosis were younger, had an increased rate of favorable risk cytogenetics, and more likely to be FLT3 and NPM1 mutated. In multivariate analysis, hyperleukocytosis was independently associated with increased relapse incidence (hazard ratio [HR] of 1.55, 95% confidence interval [CI], 1.14‐2.12; P = .004), decreased leukemia‐free survival (HR of 1.38, 95% CI, 1.07‐1.78; P = .013), and inferior overall survival (HR of 1.4, 95% CI, 1.07‐1.84; P = .013). Hyperleukocytosis retains a significant prognostic role for AML patients undergoing HSCT.     

Leukapheresis and cranial irradiation in patients with hyperleukocytic acute myeloid leukemia: no impact on early mortality and intracranial hemorrhage

To assess the role of leukapheresis and cranial irradiation in reducing the incidence of intracranial hemorrhage (ICH) and early death in patients with hyperleukocytic acute myeloid leukemia (AML) and the impact of such treatment on survival. This study retrospectively analyzed the records of 75 patients with hyperleukocytic AML who had a white cell count over 100,000/microL. All patients had de novo AML except for two with therapy-related AML. Various factors were assessed for their impact on morbidity and mortality, particularly the role of pre-induction leukapharesis and cranial irradiation. The most significant risk factors for ICH were the presence of two or more symptoms of leukostasis (odds ratios [OR] 10.6, 95% CI: 2.67-42.02; P = 0.001) and respiratory distress (OR 5.41, 95% CI: 1.44-20.32, P = 0.012). The most significant risk factors for early death were age >or= 65 (OR 4.21, 95% CI: 1.45-12.21, P = 0.008), respiratory failure (OR 3.34, 95% CI: 1.24-9.50, P = 0.018), and two or more symptoms (OR 3.50 95% CI: 1.16-10.52, P = 0.026). Neither leukapheresis nor cranial irradiation were significantly associated with a decreased incidence of ICH (P = 0.349 and 0.378, respectively). Leukapheresis had no significant influence on early death (P = 0.367). The median survival patients receiving no pretreatment was 10.50 months (range 2.58-18.42) and for those receiving pretreatment 1.50 months (range 0.10-3.16; log-rank test, P = 0.062). Leukapheresis and cranial irradiation do not improve survival or decrease the incidence of ICH in adults with hyperleukocytic AML.     

Effect of ICAM-1 and LFA-1 in hyperleukocytic acute myeloid leukaemia

Acute hyperleukocytic leukemia [AHL; WBC count >100 x 10(9)/l] is associated with a life-threatening complication. The mechanisms of hyperleukocytosis in acute myeloid leukaemia (AML) remain unclear. However, the interaction of intercellular adhesion molecule-1 (ICAM-1) and lymphocyte function-associated antigen-1 (LFA-1) plays an important role in the adhesion and migration of normal leukocytes and AML cells. Therefore, effects of ICAM-1 and LFA-1 were studied in hyperleukocytic AML. The adhesion of hyperleukocytic AML blasts and human umbilical vein endothelial cells (HUVECs) was significantly increased compared with that of blasts from non-hyperleukocytic AML (WBC < 100 x 10(9)/l). The adhesion of normal neutrophils and HUVECs treated with hyperleukocytic AML blast supernatant was increased significantly. Finally, we determined the ICAM-1 on the surface of HUVECs treated with the supernatant of hyperleukocytic AML blasts and LFA-1 on hyperleukocytic AML blasts by flow cytometry. It showed that the ICAM-1 expression on the surface of the HUVECs treated with hyperleukocytic AML blast supernatant for 24 h could be increased, and the expression of LFA-1 on hyperleukocytic AML was also increased significantly. Our data show that hyperleukocytic AML blasts stimulate the endothelium to secrete more ICAM-1 and promote their own adhesion to vascular endothelium, suggesting that ICAM-1 and LFA-1 may have a role in hyperleukocytic AML.     

Effect of ICAM‐1 and LFA‐1 in hyperleukocytic acute myeloid leukaemia

Acute hyperleukocytic leukemia [AHL; WBC count >100 × 10⁹/l] is associated with a life‐threatening complication. The mechanisms of hyperleukocytosis in acute myeloid leukaemia (AML) remain unclear. However, the interaction of intercellular adhesion molecule‐1 (ICAM‐1) and lymphocyte function‐associated antigen‐1 (LFA‐1) plays an important role in the adhesion and migration of normal leukocytes and AML cells. Therefore, effects of ICAM‐1 and LFA‐1 were studied in hyperleukocytic AML. The adhesion of hyperleukocytic AML blasts and human umbilical vein endothelial cells (HUVECs) was significantly increased compared with that of blasts from non‐hyperleukocytic AML (WBC < 100 × 10⁹/l). The adhesion of normal neutrophils and HUVECs treated with hyperleukocytic AML blast supernatant was increased significantly. Finally, we determined the ICAM‐1 on the surface of HUVECs treated with the supernatant of hyperleukocytic AML blasts and LFA‐1 on hyperleukocytic AML blasts by flow cytometry. It showed that the ICAM‐1 expression on the surface of the HUVECs treated with hyperleukocytic AML blast supernatant for 24 h could be increased, and the expression of LFA‐1 on hyperleukocytic AML was also increased significantly. Our data show that hyperleukocytic AML blasts stimulate the endothelium to secrete more ICAM‐1 and promote their own adhesion to vascular endothelium, suggesting that ICAM‐1 and LFA‐1 may have a role in hyperleukocytic AML.     

急性白血病并发颅内出血死亡23例

统计我所１９９３年１月至１９９４年８月期间急性白血病合并颅内出血死亡病例２３例，死亡原因为血小板减少，高白细胞血症、ＤＩＣ和肝损害等，表明早期死亡组主要与高白细胞血症和血小板减少有关，分析了颅内出血与ＦＡＢ分型，病情缓解与否、化疗、合并感染及贫血的相互关系，得出Ｍ３型，化疗，原发病未控制及合并感染是颅内出血的危险因素的结论。探讨了颅内出血的临床先兆表现。     

Correlation between expression of CD56/NCAM and severe leukostasis in hyperleukocytic acute myelomonocytic leukaemia

OBJECTIVE: The possible contribution of surface molecules to the development of leukostasis syndrome in hyperleukocytic acute myeloid leukaemia (AML) was assessed by routine immunophenotyping and grading of the probability of clinical leukostasis. METHODS: Fifty-three patients (23 women, 30 men, median age 59 yr) with hyperleukocytic AML [white blood count (WBC) above 50 x 10(9)/L] were graded for the probability of clinical leukostasis according to the severity of neurologic, pulmonary and other symptoms possibly caused by leukostasis using a recently published scoring system. Age, WBC, absolute blast count, haemoglobin, cytogenetic risk group, infection, relative CD56 expression and absolute count of CD56 positive blasts were analyzed in multivariate stepwise backward logistic regression analysis. RESULTS: In patients with acute monocytic leukaemia (AML M4/M5) the absolute count of leukaemic blasts expressing CD56/NCAM was highly associated with the development of symptoms graded as highly probable leukostasis and all three patients succumbing to early death were CD56 positive. Only the absolute count of CD56 positive blasts was a significant predictor of risk of severe leukostasis (P = 0.020). This was not found in AML without monocytic involvement (AML M1, M2, M3v). CONCLUSIONS: The expression of CD56/NCAM, a surface marker used in routine immunophenotyping of AML, may help to predict severe and potentially fatal leukostasis in hyperleukocytic acute myelomonocytic leukaemia. These results emphasize the usefulness of this four-stage clinical grading scale for analysing the factors, which lead to severe leukostasis in hyperleukocytic patients. We extend previous findings that the mechanisms of leukostasis are different depending on the involvement of the monocytic lineage.     

Acute myeloblastic leukemia with hyperleukocytosis: risk factors for early mortality in induction

Eighty-five patients with acute myeloblastic leukemia (AML) presenting with hyperleukocytosis (HL) were analyzed to assess morbidity and mortality in early induction. Patients who failed to achieve remission were older and more often had pulmonary leukostasis (62% vs 23%, p = .01) and hepatomegaly (54% vs 31%, p = .06) at presentation. Thirty-seven patients (44%) did not achieve complete remission (CR); 17 (54%) died early in induction therapy, 11 directly as a result of pulmonary hemorrhage with respiratory failure, while 5 had both pulmonary hemorrhage with respiratory failure and CNS hemorrhage. Early death patients were older and more often had pulmonary leukostasis (88% vs 29%, p less than .0001), hepatomegaly (71% vs 34%, p = .01), hyperbilirubinemia (60% vs 16%, p = .01) and hypofibrinogenemia (47% vs 12%, p less than .01) at presentation. Primarily for technical reasons, preinduction leukapheresis was not employed as often in this very-high-risk group as in other patients (56% vs 82%, respectively). Thus, sufficient heterogeneity exists in patients presenting with HL to define a subset of patients at particularly high risk for early mortality. Preinduction leukapheresis applied in a prospective controlled fashion should be evaluated to assess if such treatment may decrease early mortality in this group.     

Prevalence and Clinical Characteristics of Acute Myeloid Leukemia Associated with Disseminated Intravascular Coagulation

Disseminated intravascular coagulation (DIC) is one of the important complications to develop in patients with acute myeloid leukemia (AML). While acute promyelocytic leukemia (APL) is a strong risk factor for DIC, other clinical features have not been fully defined. We retrospectively analyzed 161 consecutive adult patients with de novo non-APL AML. DIC was diagnosed in 52 patients (32%); 28 patients at diagnosis and 24 soon after the initiation of induction chemotherapy. Leukocyte counts, C-reactive protein, and lactate dehydrogenase were significantly higher in the DIC+ group. Negative expressions of CD13, CD19, CD34, and HLA-DR were more prevalent in the DIC+ group. On multivariate logistic-regression analysis, variables that were independently associated with the development of DIC were high C-reactive protein, high leukocyte count, negative expressions of CD13 and HLA-DR, and cytogenetics with a normal karyotype or 11q23 abnormality. Although DIC is considered to be associated with serious morbidity and occasional mortality, we did not find any significant differences in the complete remission rate, overall or disease-free survival between DIC+ and DIC- groups. This study is the first to define the clinical characteristics associated with DIC in patients with non-APL AML, but exactly how and when DIC should be treated remains to be determined.     

急性白血病早期死亡高危因素的回归分析

采用病例对照研究，对11年上海地区27家医院2867例急性白血病（AL）住院患者早期死亡的高危同素进行Lognistic回归分析。结果：出血、感染和多脏器衰竭是AL死亡的主要原因。全反式维甲酸（ATRA）的应用，降低了急性早幼粒细胞白血病（APL）早期病死率。早期死亡的高危因素为：年龄≥60岁或≤1岁，发病时伴有明显出血、外用血白细胞＞100X109／L、BPC＜50X109／L。结论：针对上述高危因素采取相应防治措施，降低AL的早期病死率十分必要。