Response to chemotherapy in juvenile myelomonocytic leukemia and its clinical implications for survival: A retrospective registry-based study of the Korean Pediatric Hematology-Oncology Group.

Juvenile myelomonocytic leukemia (JMML) is a life-threatening myeloproliferative neoplasm. The chemotherapeutic effect on survival remains unclear, and feasible standardized response criteria are yet to be established. We aimed to evaluate the chemotherapeutic response and its effect on survival in patients with JMML.

High-Dose Busulfan-Fludarabine Conditioning and Low Alveolar Volume as Predictors of Pulmonary Complications after Allogeneic Peripheral Blood Stem Cell Transplantation in Children.

This study was conducted to investigate risk factors and predictors of infectious and noninfectious pulmonary complications (PCs) after allogeneic hematopoietic stem cell transplantation in children. We conducted a retrospective analysis of the post-transplantation PCs of 240 patients who underwent allogeneic peripheral blood stem cell transplantation (allo-PBSCT) between 2009 and 2018. Transplantation-related variables, pretransplantation baseline spirometry, body plethysmography, and CO diffusing capacity were analyzed for association with the development of infectious PCs (IPCs) and noninfectious PCs (NIPCs).

Allogeneic hematopoietic cell transplantation in patients with juvenile myelomonocytic leukemia in Korea: a report of the Korean Pediatric Hematology-Oncology Group.

Juvenile myelomonocytic leukemia (JMML) is a life-threatening myeloproliferative neoplasm. This multicenter study evaluated the characteristics, outcomes, and prognostic factors of allogeneic hematopoietic cell transplantation (HCT) in recipients with JMML who were diagnosed between 2000 and 2019 in Korea. Sixty-eight patients were retrospectively enrolled-28 patients (41.

Quantitative CT lung densitometry as an obstructive marker for the diagnosis of bronchiolitis obliterans in children.

The purpose of this study is to evaluate the quantitative diagnostic performance of computed tomography (CT) densitometry in pediatric patients with bronchiolitis obliterans (BO). We measured the mean lung density (MLD) and represented the difference of MLD in inspiratory and expiratory phases (MLDD), the ratio of the MLD (E/I MLD), and the relative volume percentage of lung density at 50-Hounsfield unit (HU) interval threshold (E600 to E950). We calculated the sensitivity, specificity, and diagnostic accuracy of the lung density indices for the diagnosis of BO.

Association between cytokine concentration kinetics and prolonged fever in febrile neutropenic children with bacteremia.

Introduction: Although prolonged fever in patients with neutropenic fever (NF) during empirical antibiotic therapy could be caused by dysregulated immune responses, its association with cytokine concentrations has rarely been investigated. This study determined the kinetics of cytokine concentrations in pediatric patients with NF and bacteremia and evaluated the impact of cytokine concentration kinetics on prolonged fever.

Methods: Concentrations of 13 cytokines were measured on the initial day of NF (Day 1) and 3 days (Day 4) and 7 days (Day 8) later in 10 patients with NF with bacteremia, and their kinetics was determined.

A Case of Disseminated Herpes Zoster Presenting as Vesicles Limited to Skin Lesions with Lymphoma Cutis Involvement.

After primary infection, varicella zoster virus (VZV) causes prolonged latent infections that may reactivate, depending on the immunologic status of the host. We present a case of VZV reactivation in a 10-year-old male patient that underwent unrelated peripheral blood stem cell transplantation (uPBSCT) for T-lymphoblastic lymphoma with lymphoma cutis lesions. This patient had a history of herpes zoster involving the right L2-5 dermatome and trigeminal V1 dermatome prior to uPBSCT.

Source Identification of Causing Six Episodes of Recurrent Sepsis in an Adolescent That Underwent Hematopoietic Stem Cell Transplantation.

Septicemia or bacteremia is one of the leading causes of death worldwide. Long-term tunneled central venous catheters (CVCs) are usually placed in children undergoing chemotherapy or hematopoietic stem cell transplantation (HSCT) for underlying hemato-oncologic malignancies. However, catheter-related complications have been reported frequently, and there is high morbidity and mortality related to catheter-line-associated bloodstream infections (CLABSIs).

Clinical characteristics and viral load patterns in children with cytomegalovirus gastrointestinal disease after allogeneic hematopoietic stem cell transplantation.

Cytomegalovirus (CMV) reactivation in allogeneic hematopoietic stem cell transplantation (allo-HSCT) causes significant morbidity and mortality. This study aimed to investigate the clinical characteristics of children diagnosed with CMV GI disease after allo-HSCT. This was a retrospective cohort study of patients <19 years old that underwent allo-HSCT during an 11-year period.

Hb-adjusted DLCO with GLI reference predicts long-term survival after HSCT in children.

This study aimed to evaluate the pretransplant diffusing capacity as a predictor of outcomes in pediatric allogeneic hematopoietic stem cell transplantation (allo-HSCT). Retrospective cohort study of 176 children followed outcomes for 5 years after allo-HSCT. We conducted an analysis of PFTs include spirometry, body plethysmography, and diffusing capacity prior to allo-HSCT.

Efficacy of low dose antithymocyte globulin on overall survival, relapse rate, and infectious complications following allogeneic peripheral blood stem cell transplantation for leukemia in children.

Antithymocyte globulin (ATG) and anti-T lymphocyte globulin (ATLG) have been widely used to prevent graft-versus-host disease (GvHD), each with distinct properties and noninterchangeable doses. However, the optimal dose of ATG in children undergoing allo-PBSCT for leukemia has not yet been established. Therefore, the impact of ATG dose on overall survival (OS), relapse, GvHD, and infectious complications was investigated.

Influence of plasma methotrexate level and MTHFR genotype in Korean paediatric patients with acute lymphoblastic leukaemia.

Whether plasma MTX concentrations and MTHFR C677T and A1298C polymorphisms could be used as a predictor of occurrence of MTX-related toxicities in Korean paediatric patients with acute lymphoblastic leukaemia (ALL) were assessed. HD-MTX related toxicities, MTHFR polymorphisms and MTX plasma concentrations following 337 HD-MTX cycles to 117 children with ALL on maintenance therapy were analyzed. A significantly higher frequency of hyperbilirubinemia ( = 0.

Serologic status and vaccine response against hepatitis B virus after allogeneic hematopoietic cell transplantation in pediatric patients.

Background: Although vaccination against hepatitis B virus (HBV) is recommended for hematopoietic cell transplantation (HCT) recipients, previous studies evaluating serologic status and immunologic response to HBV vaccination in pediatric allogeneic HCT recipients are not enough.

Objective: This study aimed to evaluate serologic status against HBV and immunologic responses to HBV vaccination in children and adolescents receiving allogeneic HCTs.

Methods: Medical records of the enrolled 61 pediatric patients < 19 years of age who received their first allogeneic HCTs were retrospectively reviewed.

Ultrasound in Hepatic Veno-occlusive Disease (HVOD) after Pediatric Hematopoietic Stem Cell Transplantation (HSCT): Comparison of Diagnostic Criteria Including the Pediatric Criteria of European Society for Blood and Marrow Transplantation (EBMT).

The European Society for Blood and Marrow Transplantation (EBMT) has recently announced new diagnostic criteria for pediatric hepatic veno-occlusive disease (HVOD) after hematopoietic stem cell transplantation (HSCT). We retrospectively inspected 97 ultrasound exams of 60 pediatric HSCT patients, and compared its diagnostic value using the Baltimore, Seattle and pediatric EBMT criteria. Nine of the ten patients who were diagnosed as HVOD only in the EBMT criteria had severe or very severe HVOD.

Seroprevalence of Hepatitis A Virus in Pediatric Patients with Hematologic Malignancies after Chemotherapy and Hematopoietic Cell Transplantation.

This retrospective study was performed to determine the seroprevalence of hepatitis A virus (HAV) in children and adolescents with hematologic malignancies after the completion of chemotherapy and hematopoietic cell transplantation (HCT). Of 97 enrolled patients, 60 (61.9%) were seropositive for HAV.

Distinct Clinical Manifestations of Thyroid Cancer After Hematopoietic Stem Cell Transplantation.

Background: The incidence of a secondary solid malignancy after hematopoietic cell transplantation (HCT) is increasing in long-term survivors.

Objective: The aim of this study was to compare the clinicopathological characteristics of HCT recipients with secondary thyroid cancer (STC), with those of non-HCT thyroid cancer patients.

Methods: We retrospectively investigated 5184 patients who received HCT between 1983 and 2016.

Respiratory Viral Infections in Children and Adolescents with Hematological Malignancies.

Background: Despite the introduction of a polymerase chain reaction (PCR) test for the diagnosis of respiratory viral infection (RVI), guidance on the application of this test and the management of RVI in immunocompromised children is lacking. This study evaluated the clinical characteristics of RVI and established strategies for the PCR test in children and adolescents with hematological malignancies.

Methods: This study included children and adolescents with underlying hematological malignancies and respiratory symptoms, in whom a multiplex PCR test was performed.

Enterococcal bacteremia in febrile neutropenic children and adolescents with underlying malignancies, and clinical impact of vancomycin resistance.

Purpose: Enterococci are a common cause of bacteremia in immunocompromised patients. Although the increase of vancomycin-resistant enterococci (VRE) makes appropriate antibiotic therapy difficult, clinical characteristics of enterococcal bacteremia and the impact of VRE infection on outcomes have rarely been reported in immunocompromised children.

Methods: We enrolled children and adolescents (< 19 years of age) with underlying malignancies who were diagnosed with enterococcal bacteremia during febrile neutropenia between 2010 and 2017.

Thyroid dysfunction in children with leukemia over the first year after hematopoietic stem cell transplantation.

Background Thyroid function in children with leukemia during the first year after hematopoietic stem cell transplantation (HSCT) was investigated. Methods The medical records of 186 subjects [111 boys and 75 girls; lymphoid=75, myeloid=111; median age at HSCT was 10.7 (0.

Clinical characteristics and outcomes of Pseudomonas aeruginosa bacteremia in febrile neutropenic children and adolescents with the impact of antibiotic resistance: a retrospective study.

Background: Although the proportion of Pseudomonas aeruginosa infections has reduced after the introduction of antibiotics with anti-pseudomonal effects, P. aeruginosa bacteremia still causes high mortality in immunocompromised patients. This study determined the clinical characteristics and outcomes of P.

Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children.

Background: Autoimmune cytopenia (AIC) is a rare complication of allogeneic hematopoietic cell transplantation (HCT). In this study, we reviewed the diagnosis, treatment and response to therapy for pediatric patients with post-HCT AIC at our institution.

Methods: Of the 292 allogeneic HCTs performed from January, 2011 to December, 2015 at the Department of Pediatrics, The Catholic University of Korea, seven were complicated by post-HCT AIC, resulting in an incidence of 2.

Varicella zoster virus infection after allogeneic hematopoietic cell transplantation in children using a relatively short duration of acyclovir prophylaxis: A retrospective study.

Although acyclovir prophylaxis against varicella zoster virus (VZV) infection for ≥1 year is recommended after allogeneic hematopoietic cell transplantation (HCT), the emergence of acyclovir-resistant viruses and adverse drug effects cannot be ignored. We investigated the cumulative incidence of VZV infection after allogeneic HCT in children receiving a shorter duration of acyclovir prophylaxis than recommended and evaluated the appropriateness of the short duration of acyclovir prophylaxis.Medical records of 217 children who received allogeneic HCT were retrospectively reviewed until a median of 25 months (range = 1-59 months) after HCT.

Clinical characteristics and outcomes of varicella zoster virus infection in children with hematologic malignancies in the acyclovir era.

Background: Although intravenous acyclovir therapy is recommended for varicella zoster virus (VZV) infection in immunocompromised children, the clinical characteristics and outcomes of VZV infection in the acyclovir era have rarely been reported.

Methods: The medical records of children diagnosed with varicella or herpes zoster virus, who had underlying hematologic malignancies, were retrospectively reviewed, and the clinical characteristics and outcomes of VZV infection were evaluated.

Results: Seventy-six episodes of VZV infection (herpes zoster in 57 and varicella in 19) were identified in 73 children.

Fatal systemic adenoviral infection superimposed on pulmonary mucormycosis in a child with acute leukemia: A case report.

Background: Although adenovirus (ADV) infection usually causes self-limiting respiratory disorders in immune competent children; severe and systemic ADV infection in children undergoing chemotherapy for leukemia has been continuously reported. Nevertheless, there has been no consensus on risk factors and treatment strategies for severe ADV infection in children undergoing chemotherapy.

Case Summary: We report a case of a 15-year-old boy with a fatal systemic ADV infection.