儿童慢性粒细胞性白血病治疗进展

儿童慢性粒细胞性白血病（childhood chronic myeloid leukemia,CCML）是一种少见的儿童获得性造血干细胞恶性克隆增殖性疾病,仅占儿童白血病的3％--5％。由于CCML罕见,诊治方法多借鉴成人CML的治疗经验,对其发病、诊断、治疗的儿童特征及其预后有待进一步追踪和探讨。本文将根据近年发表的相关文献及国外指南和推荐,就目前CCML的诊断及治疗进展进行综述。     

异基因造血干细胞移植治疗儿童慢性粒细胞性白血病的疗效分析

目的：研究异基因造血干细胞移植（allogeneic hematopoietic stem cell transplantation, allo-HSCT）治疗儿童慢性粒细胞性白血病（chronic myelogenous leukemia, CML）的治疗效果,寻找可能的影响因素,以期改善患者预后。方法：对接受allo-HSCT治疗的20例儿童CML患者,分别从年龄、性别、诊断至移植间隔时间、供受体HLA配型相合情况、移植时患儿疾病状态以及急慢性移植物抗宿主病（gost-v-host disease, GVHD）等多种因素进行疗效分析。结果：截止至随访日期,20例患者中,13例无病存活,7例死亡,其中4例死于急性重度GVHD,2例死于慢性GVHD及其并发症,1例死于移植后复发,3年总无病生存率为（64.6±1.1%）。单因素分析显示年龄是影响儿童CML治疗预后最重要的因素之一（P0.05）。多因素logistic回归分析也进一步证明仅年龄是影响预后的因素（P<0.01）。各种严重急慢性 GVHD是引起患者死亡最重要的原因。选择10位点全相合的供体进行移植治疗预后好。结论：allo-HSCT能有效治疗儿童CML,对于年龄≥10岁的CML患儿宜早期行allo-HSCT移植治疗,且尽可能选择10位点全相合的供体进行移植,积极防治GVHD,改善CML患儿移植治疗后的转归。     

儿童急性髓细胞性白血病201例临床分析

回顾性分析苏州儿童医院1998年1月至2005年12月收治的201例急性髓细胞性白血病（AML）患儿的临床资料,探讨临床特点及治疗、生存情况。[第一段]     

儿童急性髓细胞白血病诊疗建议

1．临床症状、体征：有发热、苍白、乏力、出血、骨关节疼痛及肝、脾、淋巴结肿大等浸润灶表现。     

Juvenile chronic myelocytic leukemia—report of 10 cases

Ten children (five boys and five girls) with juvenile chronic myelocytic leukemia were seen over a period of 12 years (1980–1991) at the All India Institute of Medical Sciences, New Delhi. With the exception of one who was aged 4.5 years, all children were below 4 years of age (mean age 20.4 months). The presenting features included fever, bleeding secondary to thrombocytopenia, marked hepatosplenomegaly, and skin rash. The striking hematological features were anemia, thrombocytopenia, peripheral blood monocytosis, and normoblastemia. There was no significant myeloid proliferation in the bone marrow aspirate (mean M:E = 5:1), while erythroid proliferation was prominent along with monocytosis (mean 11.2%). Fetal hemoglobin was raised in 8 of the 10 patients (mean 14.1%). Long-term survival was poor, with maximum survival being 18 months in one case. New modalities of management of this rare entity are discussed. © 1995 Wiley-Liss, Inc.     

Juvenile chronic myelocytic leukemia: experience with intensive combination chemotherapy

Six children with juvenile chronic myelocytic leukemia (JCML) with adverse prognostic features were treated with intensive combination chemotherapy similar to that utilized in patients with acute nonlymphocytic leukemia (ANLL). Despite obtaining hematologic remissions after induction therapy, clinical findings of extramedullary disease persisted. The use of intensive post-induction chemotherapy did not erradicate persistent extramedullary disease, and all patients developed hematologic relapse and progressive disease at a median of 8 months. The median survival of the treated patients was 15 months. The use of intensive ANLL therapy in poor prognosis JCML does not improve the survival rates reported with less intensive regimens but does have value in producing hematologic remissions that may be useful in preparing patients for bone marrow transplant.     

Chronic idiopathic thrombocytopenic purpura followed by chronic myelocytic leukemia

An 8-year old girl was diagnosed as having idiopathic thrombocytopenic purpura which later became refractory to steroids, splenectomy, and immunosuppressive therapy. While she was being treated for pneumococcal meningitis, 7 1/2 years later, she was found to have Philadelphia-chromosome-positive chronic myelocytic leukemia. She died 2 1/2 years after the diagnosis of leukemia. The association of autoimmune disorders and hematologic malignancies has been well-recognized. To our knowledge, there has been no previous report of chronic idiopathic thrombocytopenic purpura preceding the development of chronic myelocytic leukemia in the literature.