Abstract:
BACKGROUND:Alterations in the tumor suppressor gene TP53 have been associated with poor outcome in adult hematological malignancies. We have earlier reported an increased expression of the TP53 encoded protein p53, in bone marrow samples from pediatric patients with aggressive leukemia. Our aim was now to evaluate p53 protein expression at different time points before and after hematopoietic stem cell transplantation (HSCT) as a predictor of relapse in a group of children diagnosed with MDS, JMML and CML, and also investigate if potential alterations in expression could be correlated to mutations in TP53. PROCEDURE:Paraffin embedded bone marrow samples from 33 pediatric patients diagnosed with MDS, JMML and CML between 1997 and 2010 were collected retrospectively from time of diagnosis and pre and post HSCT. Immunohistochemistry (IHC) was performed on tissue microarrays (TMA) with antibodies to p53 and p21. DNA sequencing of exon 2-11 of TP53 was performed in 7 patients with JMML and 5 patients with MDS. RESULTS:Elevated p53 protein expression at diagnosis predicted for relapse, odds ratio (OR) 1.19 (95% CI: 1.02-1.40, p = .028). Sequencing of TP53 did not reveal any mutations in the 12 patients analyzed and p53 expression correlated positively to p21 expression indicating a functional p53/p21 protein pathway. CONCLUSION:Elevated p53 protein expression at diagnosis may be an indicator of relapse in children with MDS, JMML and CML.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Honkaniemi E,Mattsson K,Barbany G,Sander B,Gustafsson Bdoi
10.3109/08880018.2014.898723subject
Has Abstractpub_date
2014-05-01 00:00:00pages
327-39issue
4eissn
0888-0018issn
1521-0669journal_volume
31pub_type
临床试验,杂志文章abstract::At present, urinary vanillylmandelic acid (VMA) and/or homovanillic acid (HVA), metabolites of catecholamines, are the most sensitive diagnostic markers of neuroblastoma. They can be measured by the high-performance liquid chromatographic method, which has been used to screen for neuroblastoma in infants in Japan. Fro...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019709030914
更新日期:1997-11-01 00:00:00
abstract::According to the Fifth National Wilms Tumor Study (NWTS-5), tumor-specific loss of heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of patients with Wilms tumor (WT) who despite having favorable histology (FH) have a significantly increased risk of relapse and death. We aimed to find out 1p and 16q ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2015.1071902
更新日期:2015-01-01 00:00:00
abstract::In patients undergoing bone marrow transplantation cryptococcosis is rarely encountered. We report a fatal case of Cryptococcus meningitis in a 12-year-old girl with acute lymphoblastic leukemia (ALL) in second remission who had a transplant from a human leukocyte antigen (HLA)-identical unrelated bone marrow donor. T...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019709028778
更新日期:1997-09-01 00:00:00
abstract::We investigated bone marrow (BM) recovery of hematopoietic stem cells (HSC) and hematopoietic microenvironment after chemotherapy in childhood acute lymphoblastic leukemia (ALL). Twenty-nine de novo childhood ALL patients were enrolled and BM biopsy sections at diagnosis (BM0), after induction (BM1), consolidation (BM...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2019.1623354
更新日期:2019-05-01 00:00:00
abstract::A 20-year-old female developed a relapse of B-precursor acute lymphoblastic leukemia (ALL) as a mass in her left breast after 6 years of maintained continuous complete remission. No leukemic lesions were identified in other sites such as the bone marrow or cerebrospinal fluid. The relapsed leukemic cells in the breast...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010802258399
更新日期:2008-09-01 00:00:00
abstract::Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal hematological syndrome that causes a disturbance of the immune system. Overall mortality of HLH is greater than 50% and the majority of patients who die do so within the first 8 weeks of chemotherapy treatment. To find clinical parameters relating to high-ri...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2013.858198
更新日期:2014-04-01 00:00:00
abstract::Between 1983 and 2008, prenatal diagnostic procedures for identifying hemoglobinopathies were performed in 947 at-risk fetuses. Seventy-six percent of the fetuses were at risk for β-thalassemia major and 16% for sickle cell anemia; only a small percentage (7%) were at risk for compound heterozygosity of β-thalassemia ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2010.507690
更新日期:2011-02-01 00:00:00
abstract::A newborn infant presented with intraocular tumor that was clinically diagnosed as retinoblastoma and treated by exenteration of the globe. Within a few weeks however, a large abdominal mass was noted, which was found to involve the left kidney and right lobe of liver. Pathologic evaluation of the intraocular mass as ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019109033425
更新日期:1991-01-01 00:00:00
abstract::A case of a child with paroxysmal nocturnal hemoglobinuria (PNH) is characterized by an increased sensitivity of the erythrocyte to hemolytic action of complement. The widely used Ham test may not always be reliable. Recently, a panel of monoclonal antibodies has become available to detect various glycosylphosphatidyl...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019309029514
更新日期:1993-10-01 00:00:00
abstract::A case of eosinophilic meningitis 2 months before the appearance of lymphoblasts in the cerebrospinal fluid is described in a child with acute lymphoblastic leukemia (ALL). The peripheral blood showed no simultaneous eosinophilia. The child was successfully treated for her CNS relapse, and complete remission was easil...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018709141276
更新日期:1987-01-01 00:00:00
abstract::Immune thrombocytopenia (ITP) is an acute self-limited disease of childhood, mostly resolving within 6 months irrespective of whether therapy is given or not. Treatment options when indicated include corticosteroids, intravenous immune globulin (IVIG), and anti-RhD immunoglobulin. We reviewed our 32 years' experience ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2015.1040931
更新日期:2015-01-01 00:00:00
abstract::Anaplastic lymphoma kinase (ALK) inhibitors such as crizotinib and alectinib have been shown to have significant activity in ALK-rearranged non-small cell lung cancers (NSCLC). There are no data for alectinib's safety or efficacy in younger patients, though it is superior to crizotinib in adult trials. We present a 14...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2018.1541492
更新日期:2018-01-01 00:00:00
abstract::Medulloblastoma (MB) is a cancer of the cerebellum and the most common primary pediatric malignancy of the central nervous system. Classified as a primitive neural ectoderm tumor; it is thought to arise from granule cell precursors in the cerebellum. The standard of care consists of surgery, chemotherapy and age-depen...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018.2012.698372
更新日期:2012-09-01 00:00:00
abstract::Since 1981, the Children's Cancer and Leukemia Study Group (CCLSG) has developed a series of protocols for treatment of acute lymphoblastic leukemia (ALL) in childhood. In the first randomized controlled study of the 811 protocol (1981-1983) a comparison of conventional daily 6-mercaptopurine and methotrexate with a p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019709030881
更新日期:1997-01-01 00:00:00
abstract::In 33 children, 23 with acute lymphoblastic leukemia (ALL) and 10 with solid tumors, the phenotype of the enzyme adenosine deaminase (ADA) was detected in the erythrocytes by electrophoresis in cellulose acetate. In all children the ADA enzyme activity was also determined in the plasma by spectrophotometry at the onse...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019609030840
更新日期:1996-07-01 00:00:00
abstract::Invasive fungal infection continues to pose a significant threat to immunocompromised patients, with cerebral aspergillosis being among the most feared ones. The authors describe an adolescent girl with acute lymphoblastic leukemia (ALL) with subsequent acute liver failure, who developed an aspergillus brain abscess. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010500278665
更新日期:2005-12-01 00:00:00
abstract::The objective of this study was to ascertain the reactions and experiences of parents whose children were defined as false-positive cases in a research program of screening 6-month-old babies for neuroblastoma. Parents of seven of the eight infants falling into this category participated in the study. Parents of five ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019409141651
更新日期:1994-03-01 00:00:00
abstract::The aim of this study was to investigate the impact of therapy on long-term gonadal function of young people cured of childhood lymphomas and to assess whether a prepubertal state during the treatment protects the gonads from chemotherapy and/or radiotherapy late effects. Clinical evaluation, semen analysis, and endoc...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800100276415
更新日期:2000-04-01 00:00:00
abstract::Endocrine system dysfunctions are the significant complications of excessive iron overload in beta thalassemia patients. The aim of this study was to evaluate the long-term effect of chelation with deferasirox on endocrine complications. The study group consisted of children with beta thalassemia who had been evaluate...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2020.1734124
更新日期:2020-09-01 00:00:00
abstract::A 4-month old girl was diagnosed as having stage IV neuroblastoma of the right adrenal gland. Preoperative chemotherapy was given, followed by local surgical excision. Postoperatively, irradiation of the tumor bed and adjuvant chemotherapy was given for 11 months. Nine years after cessation of chemotherapy, the patien...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/088800101750476050
更新日期:2001-10-01 00:00:00
abstract::Fever and neutropenia (FN) is a common precipitant for hospitalization among children with cancer, but hospital utilization trends are not well described. This study describes national trends for hospital discharges for FN among children with cancer for the year 2012, compared with the authors' previous analysis from ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.3109/08880018.2015.1102998
更新日期:2016-02-01 00:00:00
abstract::The purpose of this study was to evaluate the feasibility and tumor response of 3 cycles of sequential high-dose chemotherapy (HDCT) in advanced pediatric solid tumor patients. Medical records of 11 children who underwent 2 consequent courses of reduced conditioning HDCT followed by final HDCT with autologous HSC infu...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/14992020903352226
更新日期:2010-02-01 00:00:00
abstract::High doses of intravenous deferoxamine via central venous line (CVL) has recently been shown to improve survival in patients with noncompliant thalassemia major (TM). The aim of this retrospective study was to evaluate the extent of CVL-related thrombosis and to determine the presence of prothrombotic factors in child...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010490457042
更新日期:2004-07-01 00:00:00
abstract::Advancements in surgery, radiotherapy, and chemotherapy have greatly improved the cures rates for children with Wilms tumor. However, a number of therapy-related late effects have been observed in long-term survivors. Generally, late complications are a consequence of treatment type and intensity; the use of radiother...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880010903019344
更新日期:2009-09-01 00:00:00
abstract::A discussion of a 5-year-old child with congenital hemolytic anemia and severe hypotonia caused by triosephosphate (TPI) deficiency is presented. The complexities in the diagnosis and management of this condition is discussed and the relevant literature is reviewed. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019809018318
更新日期:1998-11-01 00:00:00
abstract::The majority of the anemias during childhood are hypochromic and microcytic. The aim of the present study was to determine the status of α-thalassemia mutations and its association with other etiologies, such as iron deficiency anemia (IDA) and β-thalassemia trait, that are frequently seen hypochromic microcytic anemi...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2012.661831
更新日期:2012-04-01 00:00:00
abstract::Growth was studied longitudinally in 19 children who were long-term survivors after acute lymphoblastic leukemia (ALL). Of the children, 13 were girls; 6 were boys. They had all undergone a 3-year cytostatic treatment period which included vincristine, adriamycin, asparaginase, methotrexate, purinethol, and prednisone...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019209016589
更新日期:1992-07-01 00:00:00
abstract::Disaccharidases activity in the intestinal mucosa samples of rats was examined after intragastric and intramuscular methotrexate therapy. Methotrexate was given on a twice a week schedule (1 mg/kg body weight). The animals were sacrificed after 2 weeks, 1 month, and 2 months of this therapy. A statistically significan...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019009033397
更新日期:1990-01-01 00:00:00
abstract::Increased susceptibility to infection is reported in patients with beta-thalassemia major due to toxic effect of iron on neutrophil functions and reticuloendothelial system dysfunction. This study investigated the association between the neutrophil apoptosis and frequency of infection episodes, desferrioxamine treatme...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/713842280
更新日期:2003-04-01 00:00:00
abstract::Maternal education might be an important factor for the neuroblastoma risk in children, but it was conflicting. This meta-analysis was performed to evaluate the relationship between maternal education and neuroblastoma susceptibility and to explore whether maternal education was an important indicator to be associated...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,meta分析
doi:10.3109/08880018.2012.742605
更新日期:2013-02-01 00:00:00