ADEP方案双诱导加HAD方案治疗儿童急性粒细胞和急性粒单核细胞性白血病临床疗效分析

目的　分析初治急性粒细胞性白血病 (急粒 )和急性粒单核细胞性白血病 (M4)患儿用ADEP(阿糖胞苷、柔红霉素和依托泊苷 )方案双诱导加HAD(大剂量阿糖胞苷和柔红霉素 )方案的疗效。方法　总结 1991年 7月至 1999年 12月北京大学人民医院 2 6例急粒和 10例M4其中男 2 1例 ,女 15例 ,EFS(长期无病生存 ) 3年以上 ,停药 2年以上 ,中位EFS 7年 ,随访时间至 2 0 0 2年 12月。结果　 (1)用ADEP诱导治疗一个疗程完全缓解率 (CR)91% ,粒细胞白血病EFS率 5 0 % ,M4EFS率 5 7% ,中位生存时间 7年。 10例复发患儿 83%在 6～ 10个月内复发。(2 )大剂量阿糖胞苷 1g/m2 每日 2次持续 3d或 4d加蒽环类方案两者EFS率和复发率无差别 ,但在疗程中 16例用Idr(去甲氧柔红霉素 )的患儿长期存活 11例 (6 8 75 % ) ,6例不用Idr的患儿长期存活 1例 (16 7% ) ,差异有显著性。结论　 (1)用ADEP双诱导方案加HAD方案 ,在疗程中用Idr,有高的CR率和较高的EFS率。 (2 )对 10例复发病例分析 ,9例有高危因素存在 ,复发时间 80 %在CR后 6～ 10个月内 ,因此有高危因素的患儿 ,最好在CR 4～ 6个月期间行造血干细胞移植治疗     

T-cell acute lymphoblastic leukemia relapsing as acute myelogenous leukemia

We present the unusual case of a 16-year-old girl with T-cell acute lymphoblastic leukemia (ALL) with an early thymocyte immunophenotype without myeloid markers, who after 13 months of complete hematological remission relapsed as acute myelogenous leukemia (AML) with minimal differentiation and died of her disease. Whether the AML represented a relapse with lineage switch of the original immature T-cell clone or a new secondary malignancy, could not be proven due to the absence of molecular or clonal markers. This report suggests that a subset of CD7+ T-cell leukemias without mature T-cell antigens (CD4-, CD8-) are minimally differentiated and can relapse as AML.     

Acute myeloid leukemia

Acute myeloid leukemia (AML) is a heterogeneous group of leukemias that result from clonal transformation of hematopoietic precursors through the acquisition of chromosomal rearrangements and multiple gene mutations. As a result of highly collaborative clinical research by pediatric cooperative cancer groups worldwide, disease-free survival has improved significantly during the past 3 decades. Further improvements in outcomes of children who have AML probably will reflect continued progress in understanding the biology of AML and the concomitant development of new molecularly targeted agents for use in combination with conventional chemotherapy drugs.     

Acute lymphoblastic leukemia

Acute leukemia is the most common childhood malignancy, representing 30% of all cancer in American children under the age of 15 years and 12% of cancer cases in those ages 15 to 19 years old. In the United States, approximately 2500 new cases are diagnosed annually; 80% of these are acute lymphoblastic leukemia, 15% are acute myelogenous leukemia, and 5% belong to the chronic leukemia category.(1) The survival rates of children with acute leukemia have increased dramatically in the last 40 years.(2-5) The most success in outcome has occurred in acute lymphoblastic leukemia, although improvement is also being reported in acute myelogenous leukemia in the past few years. Progress comes from treatment strategy modifications on the basis of observations made in sequential large-scale therapeutic trials, an approach that serves as a paradigm for research in other malignant diseases.     

Differentiating juvenile myelomonocytic leukemia from chronic myeloid leukemia in childhood

Juvenile myelomonocytic leukemia (JMML) is a rare clonal myeloproliferative disease of early childhood. To determine the diagnostic features, appropriate treatment, and overall patient survival pertaining to JMML for children, the authors reviewed the clinical data of 16 children with JMML admitted to the National Taiwan University Hospital between 1978 and 2001. Median age at diagnosis was 2.5 years. Fever was the most common symptom at diagnosis. At initial presentation, the mean white blood count and absolute monocyte count were 30 x 10(9)/L and 4.5 x 10(9)/L, respectively. Cytogenetic analysis was performed in 14 patients, and 2 patients (14%) had monosomy 7. Another patient, with normal karyotype at diagnosis, had deletion of 7q22 at the follow-up chromosome study. Forty-seven chronic myeloid leukemia (CML) patients were also diagnosed and followed at the same hospital during the same interval period. The age, leukocyte counts, platelet counts, basophil counts, monocyte percentages on peripheral blood smears, and median survival rate showed significant differences between JMML and CML patients (P < 0.05). The median survival was 10 months and the probability of 10-month survival was 0.38 by Kaplan-Meier analysis for 12 of the 16 JMML patients who did not receive hematopoietic stem cell transplantation (HSCT). Among three patients receiving HSCT, one patient relapsed 9 months after the first HSCT and was treated successfully by a second HSCT from the same sibling donor.     

Philadelphia chromosome-positive chronic myelogenous leukemia following apparent acute lymphoblastic leukemia

The observation that Philadelphia chromosome-positive acute lymphoblastic leukemia (ALL) may progress to chronic myelogenous leukemia (CML) has been well-documented among adult patients but reported only rarely in children. In this report, we describe a pediatric patient with atypical ALL, who subsequently went on to develop classical adult-type CML. This patient is unique and important because of the unusual clinical findings that she exhibited during the "ALL" phase, namely, extreme thrombocytosis, marrow fibrosis, and persistent splenomegaly, which heralded the onset of frank CML. It is suggested that the patient with presumed, but atypical, ALL be carefully evaluated for CML presenting in lymphoblastoid crisis, and that all patients with the morphologic diagnosis of ALL undergo complete cytogenetic and immunologic marker studies to confirm the diagnosis.     

Acute lymphoid leukemia

During the past decade progress in the treatment of childhood acute lymphoblastic leukemia has slowed. A 50% to 60% cure barrier has frustrated a multitudinous array of therapeutic attempts to overcome this obstacle. With few exceptions, intensifications of induction, consolidation, or maintenance therapies have not overcome this obstacle. Current effort to break through this impasse include improved staging, biological characterization of the leukemia with newer immunodiagnostic methods, and novel approaches to therapy. The latter include continuous intensive therapy in poor prognosis patients and a combination of intensive induction consolidation and delayed intensification.     

Acute non-lymphoid leukemia

Acute non-lymphoid leukemia is a group of hematologic neoplasms which have been the subject of intensive basic and clinical research. These studies have led to a better understanding of the genetic basis of leukemia and may ultimately help establish the molecular mechanisms of malignant transformation. They also have increased our understanding of myeloid differentiation. As a result of clinical trials, we can now induce a clinical remission in a large majority of patients with acute non-lymphoid leukemia. Future studies will attempt to lessen toxicity and to maximize the response rate. Many of these advances will come from improvements in supportive care given during the periods of therapy-related marrow aplasia. The role of intensive chemotherapy to prolong remission duration and to increase the usefulness of allogenic bone marrow transplantation will be clarified during the next several years.