20例儿童急性白血病及淋巴瘤现代治疗的疗效与转归

采用强烈联合化疗方案时２０例儿童急性白血病（ＡＬ），非霍奇金氏淋巴瘤（ＮＨＬ）进行了序贯治疗及随访，总一疗程完全缓解率（ＣＲ）为９４．４％，其中急性淋巴细胞白血病（ＡＬＬ）１００％；急性非淋巴细胞白血病（ＡＮＬＬ）７５％；非霍奇金氏淋巴瘤（ＮＨＬ）１００％，１年及２年生存率（ＥＦＳ）分别为７９．９１％及５０％，总生存率为８５％，复发率１６．６６％。     

儿童急性髓系白血病靶向治疗进展

急性髓系白血病(AML)约占儿童急性白血病的15%~20%,虽然在危险度分级、分层化疗以及支持治疗等手段下AML的总体生存率较前升高,但是传统治疗下的临床疗效仍然有限,且在提高初治缓解率及减少缓解后复发方面存在局限性。近年来,随着精准医疗的不断发展,靶向治疗机制即包括AML相关信号通路的异常激活以及表观遗传修饰等研究不断深入,分子靶向药物可针对于特定的受体及目的基因等发挥作用,从而增加疗效和改善AML患者预后。     

单采血小板输注在儿童白血病和恶性淋巴瘤中的应用

为探讨儿童白血病和恶性淋巴瘤血小板显著减少期输注单采血小板的疗效,对100例急性白血病和非霍奇金氏淋巴瘤患儿输注单采血小板1单位后18h～24h测定外周血血小板计数,计算纠正计数指数(CCI)和回升率及观察临床疗效。结果99例临床出血症状改善,1例因白血病复发全身衰竭、出血死亡。输注后血小板平均升高53．41×10     

儿童急性髓系白血病的治疗进展

急性髓系白血病(acute myeloid leukemia,AML)是一组异质性疾病,约占15岁以下儿童所有确诊肿瘤的15％～ 20％,在过去的几十年里,儿童AML的长期预后取得了巨大的进展.基于AML临床和生物学特征的更加完善的危险度分层,抗白血病药物更加有效的应用以及在支持治疗方面的进展使得儿童AML治愈的可能性达到了60％左右.该文就儿童AML在化疗方案和化疗药物方面的新进展作一综述.     

靶向治疗儿童慢性粒细胞白血病临床研究

目的观察靶向治疗慢性粒细胞白血病(CML)慢性期患儿的疗效及安全性。方法选取2014年1月1日-2017年12月31日在我院血液科确诊为初发CML慢性期的患儿共5例。其中,男1例,女4例,中位年龄10(6～11)岁,均予以口服伊马替尼300mg/(m~2·d)治疗。定期观察患儿临床表现、外周血象和分子生物学指标的变化,评估疗效,同时记录治疗期间不良反应发生情况。结果5例患儿中,4例经伊马替尼规范治疗3个月后获得完全血液学反应(CHR),且在治疗12个月时均获得主要分子学反应(MMR)。1例不耐受伊马替尼,换用达沙替尼治疗1个月后获得CHR,4个月后融合基因定量检测达最佳疗效。结论伊马替尼治疗儿童CML慢性期早期疗效确切,且不良反应较轻,耐受性好,较为安全。达沙替尼可使CML患儿达到更快、更深层次的缓解,在不耐受伊马替尼的情况下,可作为二线治疗选择。     

急性白血病和淋巴瘤化疗前后巨细胞病毒感染的观察

巨细胞病毒（ＣＭＶ）是一种属于ＤＮＡ病毒条件致病病毒,所有人群都可受到感染。正常人感染后可被机体免疫系统清除,也可处于潜伏状态。当机体免疫功能低下时易发生显性感染,甚至引起严重后果［１］。急性白血病和淋巴瘤化疗后免疫功能低下,容易发生ＣＭＶ感染,对此...     

儿童初发白血病和恶性淋巴瘤血清前白蛋白测定

目的通过对前白蛋白（PA）的检测，了解其作为白血病及淋巴瘤对肝浸润的敏感指标。方法22例患儿在治疗前、后，和30例健康儿童对照组，通过免疫比浊法测定PA，并检测肝功能。结果22例治前患儿，10例（45．55％）PA下降（与对照组PA彦检验P＜0．005），5例白蛋白下降，2例谷丙、5例谷草转氨酶升高，2例总胆红素上升。治前PA异常的例数高于，肝功能值异常的例数。结论白血病和淋巴瘤对肝浸润时，PA的变化远较肝功能的变化为明显。     

Desensitization to pegaspargase in children with acute lymphoblastic leukemia and lymphoblastic lymphoma

Hypersensitivity to pegaspargase is associated with inferior survival in pediatric patients with acute lymphoblastic leukemia and lymphoblastic lymphoma. In the past year, drug‐supply shortages have led to the lack of an available alternative to pegaspargase. Rather than omit asparaginase from the treatment of acute lymphoblastic leukemia or lymphoblastic lymphoma patients with hypersensitivity to pegaspargase, we continued pegaspargase treatments for nine pediatric patients, utilizing a rapid desensitization protocol. There were no adverse events related to the pegaspargase during desensitization, and all patients who were checked had asparaginase serum levels above the threshold of 0.1 IU/mL at 7 to 14 days after pegaspargase therapy.     

强烈化疗对急性淋巴细胞白血病和非霍奇金淋巴瘤肾功能的影响

目的：了解多中心协作化疗方案（MCP方案）对急性淋巴细胞白血病（ALL）和非霍奇金淋巴瘤（NHO）患儿肾功能的影响。方法：采用自身对照法对序贯入院实施MCP化疗的ALL33例和NHL19例患儿肾功能进行多参数监测。包括血清BUN、Cr,Ccr，血、尿β2-MG、尿Alb,TRF,IgG,RBP等。结果：同化疗前比较，化疗后患者血BUN，Cr,Ccr大正常，仅9例出现镜下血尿或轻度蛋白尿；血、尿β2－MG及尿Alb,RBP显著升高（P＜0．05），而尿TRF和IgG则无明显升高（P＞0．05）。结论：MCP方案对ALL和NHL患儿的肾脏功能有一定程度的损害，以尿RBP含量各高为主要标志的肾小管损害为主，但均处于亚临床状态；是否呈可逆性改变，有待于长期随访。     

Sialic acid concentration in different stages of malignant lymphoma and leukemia in children

Sialic acid concentration in the blood serum of 104 children suffering from leukemia and malignant lymphomas was determined. A significant higher concentration of sialic acid was found at the onset of the disease, compared to the controls. In the group of children suffering from acute lymphoblastic leukemia a dependence was noted between the stage of the disease and the concentration of sialic acid in the serum. The high concentration of sialic acid at the onset of the disease, although decreasing during treatment, rose again during relapses of the disease. It seems that determination of sialic acid concentration may be useful in monitoring treatment.     

Development of diffuse large B cell lymphoma during the maintenance therapy for B-lineage acute lymphoblastic leukemia

Non-Hodgkin lymphoma (NHL) is a very rare complication of acute lymphoblastic leukemia (ALL). A Japanese boy presented with B-lineage ALL at the age of 2.5. He was treated with chemotherapy for standard-risk ALL. While he was receiving maintenance treatment 2 years and 9 months after the diagnosis of ALL, diffuse large B cell lymphoma (DLBL) was diagnosed from a biopsy of an abdominal mass. DLBL was treated by surgical resection followed by chemotherapy for 6 months. The patient has been free from the recurrence of ALL or DLBL for 16 months after the development of DLBL.     

Successful acute lymphoblastic leukemia‐type therapy in two children with mixed‐phenotype acute leukemia

Mixed‐phenotype acute leukemia (MPAL) is a rare type of leukemia expressing both myeloid and lymphoid markers. There is limited information, especially on pediatric cases. Therefore, the optimal therapeutic approach to pediatric MPAL has not been defined. Here, we report two pediatric cases of MPAL. According to the 2008 World Health Organization (WHO) classification and European Group for the Immunological Characterization of Leukemias (EGIL) criteria, patient 1 was diagnosed with overt MPAL positive for the myeloid marker myeloperoxidase (MPO), and B‐lymphoid markers. Patient 2 was diagnosed with T‐cell acute lymphoblastic leukemia (T‐ALL) using EGIL criteria. According to the 2008 WHO classification, however, patient 2 was diagnosed with overt MPAL positive for CD3, T‐lymphoid markers and MPO. We chose an ALL‐type therapy consisting of both lymphoid‐ and myeloid‐directed agents; these patients have maintained complete remission following treatment. Further information on pediatric MPAL is needed to establish an appropriate therapeutic strategy including stem cell transplantation for this rare condition.     

急性淋巴细胞白血病并中枢神经系统白血病的诊断与治疗

目的：探讨急性淋巴细胞白血病（ALL）并中枢神经系统白血病（CNSL）的诊断与治疗及影响发病和预后的因素。方法：对1990－1999年收治117例临床资料进行回顾分析。结果：CNSL发生距确诊ALL的中位数时间为8个月,高危型组发生率（54．85）明显高于标危型组（23．7％）。31例CNSL中以脑脊液（CSF）异常作出诊断远比临床症状多。CNSL治疗效果显示,大剂量氨甲喋呤＋三联鞘注＋四组（CR＋IT）相当。结论：为避免诊断假阳性造成的过度治疗,CSF仅有幼稚细胞而白细胞计数政党者诊断CNSL应慎重,HDMTX＋IT＋FC是治疗CNSL的有效措施。