TARGET based mA methylation-related genes predict prognosis relapsed B-cell acute lymphoblastic leukemia.

Purpose: The current study aims to investigate the significance of N6-methyladenosine (mA) methylationrelated genes in the clinical prognosis of childhood relapsed B-cell acute lymphoblastic leukemia (B-ALLL) patient.

Methods: Transcriptome data and corresponding clinical data on mA methylation-related genes (including 20 genes) were obtained from the Therapeutically Applicable Research To Generate Effective Treatments (TARGET) database.

Results: The bone marrow (BM) samples of 134 newly diagnosed (naive) and 116 relapsed B-ALL from TARGET were enrolled in the current study.

Coexistence of and aberration among pediatric acute lymphoblastic leukemia: case reports and a literature review.

Background: It is known that - is usually related to favorable prognosis, but aberration has been associated with poor prognosis among pediatric acute lymphoblastic leukemia (ALL). However, the outcome of coexistence of - and aberration in pediatric ALL patients is unknown. Herein, we report 4 cases of the coexistence of - and -partial tandem duplications (-PTD) in pediatric ALL patients and show the favorable outcome, which was never reported before.

Ruxolitinib inhibits the proliferation and induces the apoptosis of MLL-r ALL cells through inactivating JAK/STAT signaling pathway.

Background: The childhood patients with mixed-lineage leukemia rearrangement (MLL-r) gene have worse outcome than non-MLL, and thus often treated with high-risk chemotherapy regimens, so targeted therapy is important for this type of leukemia. This purpose of study was to explore the effects of ruxolitinib on the proliferation, apoptosis, and cell cycle of Nalm-6 cells.

Methods: In this study, human acute lymphoblastic leukemia (ALL) cell line Nalm-6 was used as the research object.

Outcome and prognostic factors of CBF pediatric AML patients with t(8;21) differ from patients with inv(16).

Purpose: To explore the outcome and prognostic factors between inv(16) and t(8;21) disrupt core binding factor (CBF) in acute myeloid leukemia (AML).

Methods: The clinical characteristic, probability of achieving complete remission (CR), overall survival (OS) and cumulative incidence of relapse (CIR) were compared between inv(16) and (8;21).

Results: The CR rate was 95.

Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study.

Purpose: To evaluate the prognostic factors and outcome for acute lymphoblastic leukemia (ALL) in children with MLL rearrangement (MLL-r).

Methods: A total of 124 pediatric patients who were diagnosed with ALL were classified into two groups based on the MLL-r status by using a retrospective case-control study method from June 2008 to June 2020.

Results: The prevalence of MLL-r positive in the whole cohort was 4.

Analysis of clinical characteristics and prognostic factors associated with EBV-associated HLH in children.

Purpose Our aim is to analyze the clinical characteristics and prognostic factors of Epstein-Barr (EB) virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children. Methods Children with newly diagnosed HLH were retrospectively analyzed. Results Finally, a total of 95 children with HLH were enrolled in this study, including 43 (45.

Poor outcome of pediatric patients with acute myeloid leukemia harboring high FLT3/ITD allelic ratios.

Activating FLT3 mutations are the most common mutations in acute myeloid leukemia (AML), but the optimal threshold of FLT3/ITD allelic ratio (AR) among pediatric AML patients remains controversial. Here, we present the outcome and prognostic significance of FLT3/ITD AR analysis among pediatric patients with AML from the TARGET dataset. Applying fitting curve models and threshold effect analysis using the restrictive cubic spline function following Cox proportional hazards models identifies the cut-off value of 0.

CEBPA are independent good prognostic factors in pediatric acute myeloid leukemia.

To evaluate the outcome and prognostic significance of CEBPA mutations among pediatric acute myeloid leukemia (AML) from TARGET dataset. A total of 1803 pediatric patients who were diagnosed with AML were classified into two groups based on the CEBPA status by using a retrospective cohort study method from September 1996 to December 2016. The incidence of CEBPA mutations was 18%.

DNA index as prognostic factor in childhood acute lymphoblastic leukemia in the COG-TARGET database.

Background: This study was aimed to evaluate the value of DNA index(DI) among pediatric acute lymphoblastic leukemia (ALL) treated on Children's Oncology Group (COG) protocols between 2000 and 2015.

Methods: Retrospective study were analysis among pediatric ALL patients from the TARGET dataset.

Result: Totally, 1668 eligible pediatric patients were enrolled in this study.

Eltrombopag as first-line treatment for thrombocytopenia among paediatric patients after allogeneic haematopoietic stem cell transplantation.

Aims: The purpose of this study is to examine the safety and efficacy of eltrombopag as first-line treatment for thrombocytopenia among paediatric patients after haematopoietic stem cell transplantation (HSCT).

Methods: Forty-three childhood patients with thrombocytopenia after HSCT who received eltrombopag were retrospectively analysed.

Result: Eltrombopag was began at the median of 27 days after HSCT and lasted for 24 days.

[Clinical Analysis of 164 Children of Blood Disease Complicated with Invasive Fungal Disease].

Objective: To investigate the clinical characteristics, prevention and treatment of invasive fungal disease (IFD).

Methods: The clinical data of 164 patients who met the diagnostic criteria of IFD in our center from January 2012 to January 2015 were retrospectively analyzed. The incidence, clinical characteristics, related factors, treatment methods and prognosis were analyzed.

Diagnosis and treatment of juvenile myelomonocytic leukemia.

To investigate clinical features, diagnosis, treatment strategies and prognosis of juvenile myelomonocytic leukemia (JMML). The clinical data of 21 patients with JMML who were diagnosed in our hospital from January 2013 to May 2018 were retrospectively analyzed. Among the 21 children with JMML, 16 were male and 5 were female.

The early diagnostic value of serum galactomannan antigen test combined with chest computed tomography for invasive pulmonary aspergillosis in pediatric patients after hematopoietic stem cell transplantation.

Objective: The purpose of our study was to evaluate the diagnostic value of serum galactomannan antigen (GM) testing combined with chest computed tomography (CT) of invasive pulmonary aspergillosis (IPA) in pediatric patients after hematopoietic stem cell transplantation.

Methods: A retrospective nested case-control study was conducted in the identifying IPA among pediatric patients.

Results: A total of 141 eligible pediatric recipients with febrile neutropenia were enrolled in this study.

Combination antifungal treatment for invasive fungal disease after hematopoietic stem cell transplantation in children with hematological disorders.

Background: Invasive fungal disease (IFD) has a poor prognosis in children with hematological disorders after hematopoietic stem cell transplantation (HSCT). We assessed if drug combinations with different targets may improve the outcome.

Methods: Retrospective study to assess the outcome of combination antifungal therapy (CAT) for proven-probable IFD (PP-IFD) in children with hematological disorders after HSCT from January 2008 to June 2018.

[Etiological Study of Late Onset Hemorrhagic Cystitis after Allogeneic Hematopoietic Stem Cell Transplantation in Children].

Objective: To prospectively study the correlation BKV with the occurrence and development of late onset hemorrhagic cystitis (LOHC) after allogeneic hematopoietic stem cell transplantation(allo-HSCT).

Methods: The clinical data of a total of 276 patients with allo-HSCT in our department between January 1998 and March 2016 were analyzed ratrospectvely. Quantitative Real-time PCR assay was used to prospectively monitor the BKV DNA load of the urine and plasma for 23 patients accepting allo-HSCT from August 2015 to March 2016.

[Efficacy and Safety of MSC Infusion in Treatment of Children with Refractory LOHC: A Clinical Study].

Objective: To study the curative effect and safety of menchymal stem cell infusion in treatment of children with refractory late-onset hemorrhagic cystitis(LOHC) after allogeneic HSCT.

Methods: Thirty cases of children with refractory LOHC after allo-HSCT in our department between December 2010 and July 2016 were analyzed retrospectively, out of 30 cases 7 received MSC treatment. The used MSC of all were four-to-five generation MSC from bone marrows of third party donors, and were infused into patients with (1.

[Clinical Efficacy of NOPHO-AML 2004 Regimen for Treatment of Children with Acute Myelocytic Leukemia (Non-M3)].

Objective: To investigate the efficacy and safety of NOPHO-AML 2004 chemotherapy regimen for treatment of children with acute myelocytic leukemia(non-M3).

Methods: Thirty-three patients aged 1-13 with acute myelocytic leukemia (non-M3) were diagnosed from January 2013 to June 2017. FAB typing showed that 1 case in M0, 4 cases in M1, 12 cases in M2, 5 cases in M4, 8 cases in M5, 1 case in M6, and 2 cases in M7; Risk stratification showed that: 19 cases in standard risk, and 14 cases in high risk.

Atrial septal defect in a patient with congenital disorder of glycosylation type 1a: a case report.

Background: Atrial septal defect often become more severe when encountered in genetic syndromes. Congenital disorder of glycosylation type 1a is an inherited metabolic disorder associated with mutations in PMM2 gene and can affect almost all organs. Cardiac abnormalities vary greatly in congenital disorder of glycosylation type 1a and congenital heart defects have already been reported, but there is little knowledge about the effect of this inherited disorder on an existing congenital heart defect.

X-linked Hyper-IgM Syndrome: A Phenotype of Crohn's Disease with Hemophagocytic Lymphohistiocytosis.

X-linked hyper-immunoglobulin M (IgM) syndrome is characterized by recurrent infections, low or undetectable levels of IgG and IgA, and normal to increased serum IgM, and is also rare. It is associated with mutation in the gene encoding CD40 ligand. This study aimed to describe the first international report of hemizygous CD40LG c.