ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Transient leukemia (TL or transient myeloproliferative disorder) occurs in approximately 10% of newborn infants with Down syndrome. The disorder is characterized by the presence of megakaryoblasts in the peripheral blood; most cases resolve spontaneously within the first 3 months of life, and the child is well thereafter. However, there are cases in which a severe, potentially lethal form of disease develops, manifesting as hepatic fibrosis or cardiopulmonary failure. Hitherto, the incidence of these severe forms of the disease has not been reported. A prospective study of TL was conducted by the Pediatric Oncology Group (POG Study 9481) in which 48 children with TL were identified. Life-threatening disease occurred in nine patients (19%); seven had hepatic fibrosis and two had cardiopulmonary failure. Five children died of the disease within the first 3 months of life, none of whom received antileukemic therapy. One patient died on day 31 after receiving minimal therapy within 1 day of death. Three children received low-dose cytosine arabinoside (Ara-C) (0.4–1.5 mg/kg every 12 hours for 5 or 7 days). In all these patients, the disease resolved. It is concluded that potentially lethal disease is relatively common in TL, and the available evidence suggests that these diseases are responsive to low-dose Ara-C therapy.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

The most common form of leukemia in Down syndrome patients is megakaryoblastic leukemia. There are two forms of the disease. Transient leukemia (TL) is a form of megakaryoblastic leukemia that occurs in newborns with Down syndrome and usually disappears spontaneously within the first 3 months of life. Acute megakaryoblastic leukemia (AMKL) occurs in Down syndrome children within the first 4 years of life and is fatal without treatment. The megakaryoblasts of TL and AMKL are indistinguishable by light and electron microscopy; yet, TL is benign and AMKL is malignant. One of the hallmarks of many malignancies is the expression of telomerase. It is therefore hypothesized that the transient, benign form of megakaryoblastic leukemia (TL) would not contain telomerase activity, whereas telomerase would be demonstrable in the malignant form of the disease. Telomerase activity was determined in the blood and/or bone marrow aspirates in 29 cases of AMKL and 34 cases of TL. The authors found telomerase activity in 15 of 29 (52%) cases of AMKL and in only 4 of 34 (12%) cases of TL (P< 0.001). Furthermore, three of the four telomerase-positive TL cases were particularly severe, of which two were fatal. Telomerase activity is found frequently in the leukemic cells of the malignant form of megakaryoblastic leukemia but rarely in the benign form of the disease (TL). Observations provide evidence that telomerase may be a critical factor for the malignant conversion of leukemic cells.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

BackgroundPhiladelphia chromosome–positive chronic myelogenous leukemia (CML) is a rare disease in children, and the optimal therapy is not clearly defined in these patients when a human leukocyte antigen-identical donor is not available. The present work focuses on the therapeutic efficacy and the toxicity of interferon (IFN) &agr;2b in combination with cytosine arabinosine (Ara-C) in patients younger than age 18 years enrolled in the randomized trial CML 91, which compared the efficacy of IFN and cytosine arabinoside (Ara-C) with IFN alone in 810 patients with CML in the chronic phase.Patients and MethodsTwelve patients younger than age 18 years were enrolled in the randomized trial CML 91. Hydroxyurea and IFN (5 million units/m2, once a day) were given as initial treatment in all patients. After randomization, six patients received IFN (5 million units/m2, once per day) and Ara-C (20 mg/m2for 10 days each month) (IFN plus Ara-C group), and six patients received IFN alone (5 million units/m2once per day) (IFN group).ResultsSix months after the beginning of the treatment, a complete hematologic response was obtained in all the patients in the IFN plus Ara-C group and in four patients in the IFN group. A major cytogenetic response was observed in three patients in the IFN plus Ara-C group and in two patients in the IFN group. Five patients from the IFN group who crossed over to receive Ara-C did not experience additional hematologic toxicity. Three patients in the IFN plus Ara-C group and two from the IFN group are alive, in major cytogenetic response, with a follow-up of 18 to 48 months.ConclusionThe combination of IFN and Ara-C induces complete hematologic and major cytogenetic responses and is well tolerated in patients younger than age 18 years with CML. This combination may offer an alternative to bone marrow transplantation in children in the chronic phase of CML without a histocompatible donor.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

PurposeChildren with malignancies in whom life-threatening complications develop are traditionally considered as having a grim prognosis. Clinical predictors of short-term outcome for rational triage to pediatric intensive care units (PICU) were retrospectively assessed.Patients and MethodsThe records of 94 children consecutively admitted to the PICU at the authors' institution between January 1989 and January 1999 were reviewed, and predictors of 30-day mortality rates were delineated using stepwise logistic regression.ResultsThe children's mean age was 7.3 years (range, 2–21). Their diseases included hematologic malignancies 45 (48%), extracranial solid tumors 21 (22%), and intracranial tumors 28 (30%). The overall 30-day survival rate was 66%. Mortality was highest among children admitted for respiratory failure (40%). High mortality was also found for those with circulatory collapse (33.3%) and neurologic deterioration (31%). The admitting pediatric risk of mortality score (PRISM) among the survivors was 6.6 ± 1.3, compared with 15.2 ± 3 among nonsurvivors (P< 0.01). The number of organ system failures was higher among the nonsurvivors on admission (P< 0.001). The need for ventilatory or inotropic support corresponded to worse outcome (P< 0.001 orP< 0.01, respectively). Overall, 36 (38%) of the children had sepsis during their PICU stay, with a mortality rate of 50% compared with 24% among nonseptic children (P< 0.01). Sepsis present on admission was later correlated with the development of organ system failure (P< 0.01).ConclusionsNew trends in therapeutic approaches to children with malignancies can clearly improve outcome. The high (66%) survival rate justifies policy of early admission to the PICU of children in whom signs of multiorgan involvement start to develop, as reflected by high PRISM and the need for ventilatory or inotropic support. Further refinement of reliable clinical predictors of survival will enable better triage of these children to the PICU for possible prevention of systemic complications and reduction of mortality rates.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

BackgroundIn osteosarcoma, as in other tumors, the presence of metastases at presentation is generally considered a consequence of late diagnosis. To verify this, the authors investigated whether there was a relationship between the stage of the disease at presentation and several clinical and pathologic characteristics, including the interval between the onset of first symptoms or signs and the final diagnosis.Patients and MethodsOne thousand seventy-one patients with high-grade osteosarcoma of the extremity were observed between 1980 and 1999. Of these, 891 had a localized tumor and 180 had metastases at the time of diagnosis.ResultsCompared with patients with localized disease, patients with detectable metastases at the time of diagnosis had higher serum levels of alkaline phosphatase, larger primary lesions, and tumors often located in the femur and humerus. In terms of time to diagnosis, the interval between the onset of first symptoms and the final diagnosis was significantly shorter in patients with metastases than in patients with localized tumor. This surprising finding probably reflects a more rapid growth of the tumor.ConclusionsThese results suggest a different biologic phenotype and aggressiveness of the tumor in a subgroup of patients and that the stage of the disease at presentation depends more on the properties of these tumors than on late diagnosis.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

PurposeTo test the hypothesis that poor response to chemotherapy in patients with bilateral Wilms tumor may be associated with the appearance of rhabdomyomatous histology, suggesting a differentiation response.MethodsTwenty-six patients with bilateral Wilms tumor were treated at the authors' hospital between 1985 and 1995. Radiologic response to presurgical chemotherapy was assessed, and postsurgery histology was reviewed.ResultsThere was a significant association between rhabdomyomatous differentiation in postchemotherapy surgical specimens and poor radiologic response. Poor response did not, however, necessarily mean poor outcome: of 11 patients with rhabdomyomatous differentiation, 7 are alive and disease-free, 2 died of complications, and only 2 died of uncontrolled Wilms tumor.ConclusionsRhabdomyomatous differentiation in postchemotherapy bilateral Wilms tumor is associated with poor radiologic response. This observation may indicate a differentiation response rather than an absolute failure of response to chemotherapy. Clinical measures other than tumor volume are needed to distinguish between tumors that respond to chemotherapy but do not shrink, and those that genuinely do not respond.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Anemia is a hematologic abnormality commonly discussed during the treatment of childhood cancer, but its incidence has not been previously reported. As the basis for determining the incidence of anemia, this retrospective review of medical records combined databases containing the records of all patients 1 to 18 years of age with newly diagnosed neuroblastoma, rhabdomyosarcoma, Hodgkin disease, Ewing sarcoma, or osteosarcoma from two pediatric oncology centers. Data from 405 patients were included in the analysis of hemoglobin at the time of diagnosis. Across diagnoses, 51% to 74% of patients were anemic using the Centers for Disease Control and Prevention age-and sex-specific values to define anemia. The long-term complications of anemia in children with cancer are unknown. Further investigation of the clinical significance of anemia, including its impact on quality of life, is warranted.

ISSN:1077-4114    

出版商:OVID    

年代:2002

数据来源: OVID