ABSTRACT
ObjectivesTo retrospectively analyze the clinicopathological features of neuroblastoma (NB) and investigate the signiifcance of abnormality ofN-myc and anaplastic lymphoma kinase (ALK) gene copy number change as well asALKmu-tations in NB.Methods Eighty-three NB patients were collected and classiifed into different subgroups according to the clinical stage and histology. Fluorescence in situ hybridization (FISH) was performed to detect the abnormalities ofN-mycandALK genes. The extracted DNA was ampliifed by PCR and sequenced to investigate the point mutations of theALK gene. Follow-up data were collected and survival analysis was performed.ResultsFISH detection showed that the aberration ofN-mycgene copy number presented as gain and ampliifcation. The aberration ofALK gene presented as point mutation and gain. It was shown that 17 cases had the abnormality of bothN-myc andALK gene. Survival analysis showed that the prognostic factors included the clinical stage, age and abnormality ofN-myc genes.ConclusionDetection ofN-myc andALK abnormality in NB would be helpful for evaluating the prognosis and providing theoretical basis forALK target therapy.
ABSTRACT
Retinoid X receptor (RXR) acts as ligand-dependent transcription factors playing an important role in regulating a serial of physiological processes,such as embryo development and organ homeostasis.At the molecular level,RXRs exert their functions by inter-activating with multiple signal pathways to regulate target gene expression which control cell growth,differentiation,survival and death.The interference in the network of RXR and other signal pathways has turned RXR into an attractive drug target.
ABSTRACT
Objective To analysis the cause of primary graft failure of unrelated cord blood transplantation with high-dose of CD34 + cells in treatment of acute myelocytic leukemia (AML) /myelodysplastic syndrome (MDS).Methods A 4-year-old girl was diagnosed AML/MDS at the Department of Pediatric Hematology and Oncology of West China Second University Hospital of Sichuan University. She presented completely remission after induction and consolidation chemotherapy.She received unrelated partially human leukocyte antigen (HLA)-mismatched cord blood transplantation.We investigated the treatment outcomes of UCBT and associated complications.Results The patient suffered primary graft failure and then received secondary haploidentical hematopoietic stem cell transplantation (HSCT)from her mother.However,she suffered fatal multiresistant Acinetobacter spp septicemia.She died due to respiratory failure on 7 d after the second transplantation.Conclusions In this case,hematopoietic stem cells with high dose of CD34 + cells could not overcome the risk of primary graft failure and HLA disparity.The patient's primary graft failure was associated with platelet transfusion refractoriness and potent immunologic dysfunction,especially the anti-HLA donor specific antibodies before unrelated cord blood transplantation.