A Novel Missense Mutation Affecting the N-terminal Domain of SAP Protein in X-linked Lymphoproliferative Disease
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Letter to the Editor Source Type: research

Treatment of Recurrent Refractory Pediatric Pre-B Acute Lymphoblastic Leukemia Using Inotuzumab Ozogamicin Monotherapy Resulting in CD22 Antigen Expression Loss as a Mechanism of Therapy Resistance
Conclusion: To our knowledge, this is the first published case of CD22 expression loss as a mechanism of therapy resistance for inotuzumab ozogamicin. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Acquired Pure Red Cell Aplasia and Acquired Amegakaryocytic Thrombocytopenia Associated With Clonal Expansion of T-Cell Large Granular Lymphocytes in a Patient With Lipopolysaccharide-responsive Beige-like Anchor (LRBA) Protein Deficiency
Acquired pure red cell aplasia and acquired amegakaryocytic thrombocytopenic purpura are rare in children. Similarly, clonal expansion of T-cell large granular lymphocytes is infrequently seen in pediatrics. Lipopolysaccharide-responsive beige-like anchor (LRBA) protein deficiency is a recently described immunodeficiency syndrome that has been associated with inflammatory bowel disease and autoimmune phenomena such as Evans syndrome. Here, we describe a patient with LRBA deficiency who developed acquired pure red cell aplasia and acquired amegakaryocytic thrombocytopenic purpura associated with expansion of clonal T-cell l...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

An 18-Year-Old Male With X-linked Lymphoproliferative Syndrome Type 1 Who Developed Primary Central Nervous System Lymphoma 6 Months After Primary Epstein-Barr Virus Infection
We report the case of an 18-year-old male who developed hemophagocytic lymphohistiocytosis (HLH) with neurologic complications after primary Epstein-Barr virus (EBV) infection and subsequently developed EBV-related central nervous system lymphoma (CNSL). Given the vulnerability to EBV, he was finally diagnosed with XLP1 and treated with whole-brain irradiation along with chemotherapy and subsequent allogeneic hematopoietic stem cell transplantation from a SH2D1A wild-type sibling donor. Although the prognosis for CNSL is generally dismal, reconstitution of the immune system from a normal donor contributed to the patient re...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Long-term Complications and Management of Gastrointestinal Bleeding in Multifocal Lymphangioendotheliomatosis
We report a case of multifocal lymphangioendotheliomatosis with thrombocytopenia in a male infant with isolated GI involvement, diagnosed when he was 3 months old. The patient was managed with daily aminocaproic acid, octreotide drip, and corticosteroids for 13 months after diagnosis; he had complete resolution of symptoms by 2 years of age and showed adequate height and gain by 5 years of age. This case adds to the paucity of data in the literature pertaining to the disease’s phenotypic variability, long-term clinical course, and management of GI bleeding. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Not All Pediatric Bladder Masses are Rhabdomyosarcoma: Report of a Rare Case With Review of the Literature
We report the third case of urinary bladder leiomyoma arising in a 6-year-old boy with a short review of the literature. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Thrombospondin-1 and Vitamin D in Children With Sickle Cell Anemia
Background: Thrombospondin-1 (TSP-1) and 25-hydroxyvitamin D (25-OHD) play significant roles in the pathogenesis of sickle cell anemia (SCA). TSP-1 enhances cellular adhesion/inflammation, hence contributing to vaso-occlusive crisis (VOC); vitamin D, in contrast, retards inflammation and may lower rate of pain episodes. We determined serum levels of TSP-1 and 25-OHD in Nigerian children with SCA and their matched hemoglobin AA controls; and assess the relationship between the 2 biomarkers. Methods: In total 90 children (32 SCA in steady state, 30 SCA in VOC, and 28 HbAA controls) were studied. Serum TSP-1 and 25-OHD l...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Activated Phosphoinositide 3 Kinase Delta Syndrome (APDS): A Primary Immunodeficiency Mimicking Lymphoma
Malignant or nonmalignant lymphoproliferative disorders together with repeated ear, nose, and throat infections should strongly motivate immunologic investigations. Indeed, we report a 7-year-old patient with a history of persistent abdominal symptoms along with recurrent ear, nose, and throat infections, who presented with intra-abdominal masses highly suggestive of a diagnostic of lymphoma, and who was diagnosed with activated-PI3K-delta syndrome, a recently described primary immunodeficiency prone to lymphoproliferation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Wilms Tumor Associated With the 9q22.3 Microdeletion Syndrome: 2 New Case Reports and a Review of The Literature
Conclusions: The 9q22.3 microdeletion syndrome should be considered at diagnosis of WT in children with dysmorphic features. Conversely, patients with a known 9q22.3 deletion syndrome should be considered for a WT predisposition surveillance program, especially those with overgrowth. The management should be individualized and given the excellent prognosis, and the unknown future risk of metachronous disease or other malignancy, the surgical approach should be carefully considered. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

The Management of Pediatric Oncology Inpatients With Oral Mucositis
The purpose of the study was to investigate the management of chemotherapy-induced oral mucositis (OM) in pediatric patients. A total of 68 separate episodes of OM were assessed in 47 children who had received chemotherapy. The severity of the child’s OM was assessed using 2 scales, and relevant clinical information was collected. The mean onset time of OM was 8.4 days (±4.0), with a median duration of 7.0 days (4.0, 10.5), with median admission of 7.0 days (4.5, 13.5). The overall adherence to an oral health protocol was 59%, which decreased with more severe OM. A third of patients used chlorhexidine mouthwas...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Outcome of Relapsed Pediatric Patients After Second Allogeneic Hematopoetic Stem Cell Transplantation: A Retrospective Study From a Single Institution
We report the outcome of 18 pediatric patients after failure of HSCT2. The 2-year overall survival was 26% (95% confidence interval [CI], 6-47). The lymphoid malignancies were associated with better survival (40% [95% CI, 12-68]) than myeloid malignancies (0%, P=0.002), together with time to relapse after the HSCT2 (≥5 mo: 44% [95% CI, 12-76] vs. 0% for patients who relapsed within 5 mo from HSCT2, P=0.005), other factors such as sex, donor type, conditioning regimen, and graft versus host disease prophylaxis did not have statistical significance. When the multivariate analysis was carried out, 2 independe...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Pediatric Patients With SHH Medulloblastoma Fail Differently as Compared With Adults: Possible Implications for Treatment Modifications
Purpose: The purpose of this work was to study the diversity of sonic hedgehog (SHH) medulloblastoma across different age groups with an emphasis on patterns of relapse. Methods: All data for the study were obtained through review of medical records, imaging, radiation charts, treatment planning, and chemotherapy details. Results: Sixty-three patients with SHH medulloblastoma were identified from a prospectively maintained database and classified into 3 groups—infantile: ≤3 years (i-SHH, n=11); pediatric:>3 to (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Gram-negative Bacteremia in Children With Hematologic Malignancies and Following Hematopoietic Stem Cell Transplantation: Epidemiology, Resistance, and Outcome
Gram-negative rod (GNR) infections adversely affect the outcome of patients with malignancies and following hematopoietic stem cell transplantation (HSCT). This retrospective observational study aimed to describe the epidemiology, outcome, and resistance patterns of GNR bacteremia in children with hematologic malignancies (HM) and after HSCT during the period spanning from 2010 to 2014 in a tertiary children’s hospital. A total of 270 children were included in the analysis; 65 (24%) developed 85 episodes of GNR bacteremia; the rate was 36/122 (29.5%) in post-HSCT and 29/178 (16.3%) in HM patients (P30 days in the las...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Nucleated Red Blood Cells in Children With Sickle Cell Disease Hospitalized for Pain
This study examined the relationship between nucleated red blood cell (NRBC) counts during hospitalization for VOE and development of ACS or transfusion requirement among children with SCD. Retrospective chart review was performed for 264 encounters of patients with SCD hospitalized for uncomplicated VOE who had NRBC count data at admission during a 5-year period. Multivariable logistic regression analysis was conducted to determine the relationship of admission and change in NRBC ([INCREMENT]NRBC) to ACS/transfusion requirement. Overall, 44 of 264 (16.7%) encounters resulted in ACS, transfusion, or both. Admission NRBC wa...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

A New Homozygous Mutation (c.393-394del TA/c.393-394del TA) in the NT5C3 Gene Associated With Pyrimidine-5′-Nucleotidase Deficiency: A Case Report
Pyrimidine-5-nucleotidase (P5′N-1) deficiency is a rare nonspherocytic hemolytic anemia due to pyrimidine nucleotide deposition within erythrocytes. This rare erythrocyte disorder shows autosomal recessive inheritance with mutation of the pyrimidine-5′-nucleotidase gene, which is localized on 7p15-p14. Consanguinity of parents increases the probability of disease with novel mutations. Here, we report a 12-year-old boy with a delayed diagnosis of P5′N deficiency whose parents were consanguineous. He had a hemoglobin level of 7.5 g/dL, mean corpuscular volume of 93 fL, 7% reticulocyte, and lac...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Separating Lymphoblastic Lymphoma Involving Marrow From Lymphoblastic Leukemia: It is Time to Update the CutOff Number
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Letters to the Editor Source Type: research

Primary Cutaneous Diffuse Large B-Cell Lymphoma With KMT2A-MLLT3: An Infantile Case Study
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Letters to the Editor Source Type: research

Cryptic ETV6-ABL1 Fusion and MLL2 Truncation Revealed by Integrative Clinical Sequencing in Multiply Relapsed Acute Lymphoblastic Leukemia
This report demonstrates the exciting potential of integrative clinical sequencing in identifying previously undetected actionable findings leading to improved outcomes in pediatric oncology patients. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Bone Marrow-derived CD8+ T Cells From Pediatric Leukemia Patients Express PD1 and Expand Ex Vivo Following Induction Chemotherapy
Adoptive cell therapy (ACT) of chimeric antigen receptor T cells has demonstrated remarkable success for the treatment of pediatric B-cell leukemia. For patients who are not candidates for chimeric antigen receptor T-cell therapy, ACT using tumor antigen-experienced polyclonal T cells may be a treatment option. Since leukemic blasts reside in the bone marrow and bone marrow is a preferred site for homeostatic proliferation of cytotoxic memory CD8+ T cells, we hypothesized that bone marrow would be a source of activated T cells. The aim of this study was to determine the feasibility of using bone marrow-derived T cells foll...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Major Issues of Care in Thalassemia Major Children Refugees
We report the case of 2 Syrian 10-year-old twin girls who arrived to France with numerous and severe complications of βTM: hemochromatosis, alloimmunization, hypopituitarism, osteopenia… Their clinical management, which led to successful vital and functional improvement, is reported in this article. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Outcome of High-risk Langerhans Cell Histiocytosis (LCH) in Egyptian Children, Does Intermediate-dose Methotrexate Improve the Outcome?
In conclusion, idMTX is associated with better EFS. DP on induction remains of dismal prognosis in relation to disease REA afterwards. Risk stratification should highlight the role of trihemopoietic cytopenias, hepatic dysfunction, tri RO+, central nervous system risk site, and lung association. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Severe ABO Hemolytic Disease of the Newborn Requiring Exchange Transfusion
ABO incompatibility (ABOi), the most common cause of hemolytic disease of the newborn (HDN), is nearly always mild and treatable with phototherapy. Reports of ABOi HDN requiring neonatal exchange transfusion are extremely rare since the inception of modern guidelines. Here, a case of ABOi HDN clearly met criteria for exchange transfusion. An O-positive African American mother delivered a B-positive neonate that quickly developed hyperbilirubinemia. The neonatal DAT was positive from anti-B and anti-A,B, and maternal IgG titer was 1024. Double volume exchange transfusion resulted in a favorable outcome. Given early discharg...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Intrapelvic Retroperitoneal Synovial Sarcoma in a 15-Year-Old Adolescent Girl: A Case Report and Review of the Literature
Synovial sarcomas are a rare subtype of soft tissue sarcomas mostly located in the lower extremities. The authors report a case of synovial sarcoma in a 15-year-old adolescent girl with several unusual features including age, intrapelvic retroperitoneal location of the primary tumor, and presentation with right abdominal tenderness and compression of the iliac vessels with thrombosis of the right iliac and femoral vein. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Catastrophic Delayed Hemolytic Transfusion Reaction in a Patient With Sickle Cell Disease Without Alloantibodies: Case Report and Review of Literature
While packed red blood cell (PRBC) transfusion therapy is a mainstay in the treatment of certain patients with sickle cell disease (SCD) and the standard of care for preoperative management, there are associated risks. Delayed hemolytic transfusion reaction (DHTR) is a risk of PRBC transfusion occurring 2 to 20 days from transfusion and typically presents with severe pain characteristic of vaso-occlusive crisis, fever, and hemolytic anemia. DHTRs are uncommon, occurring in only 4% to 11% of transfused patients with SCD, but may be catastrophic in nature with progression to multiorgan failure within hours. Here, we describe...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Immune Thrombocytopenia in a Child With Refractory Langerhans Cell Histiocytosis Following Cladribine Containing Therapy
In this report, we present a young infant with multisystem Langerhans cell histiocytosis, who after cladribine and cytarabine salvage treatment developed immune thrombocytopenia (IT). On review of the literature, there were no previous reports of Langerhans cell histiocytosis–associated IT. Treatment of the IT with intravenous immunoglobulin and oral corticosteroids was unsuccessful. Eltrombopag, in combination with a 4-day course of dexamethasone was commenced as second-line therapy. Platelet recovery occurred 10 days after initiation of eltrombopag. The immune thrombocytopenia remains in long-term remission despite...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Asymptomatic Pulmonary Artery Embolization by a Port-a-Cath Fragment in a Child With Acute Lymphoblastic Leukemia
Broken catheter embolism is a rare but often fatal complication of implantable venous access devices. Prompt removal is key to avoiding an adverse outcome. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Radiology Corner Source Type: research

Bone Marrow Failure in Fanconi Anemia: Clinical and Genetic Spectrum in a Cohort of 20 Pediatric Patients
Prognostic refinement in Fanconi anemia (FA) is needed, especially when considering allogeneic hematopoietic stem cell transplantation (HCT). We studied 20 children with FA and bone marrow failure from a single center. According to Hôpital Saint-Louis risk classification for FA, patients were classified in stage A (no or mild cytopenia/dysplasia), B (single non–high-risk cytogenetic abnormality), C (severe cytopenia and/or significant dysplasia and/or high-risk cytogenetic abnormality), and D (myelodysplastic syndrome with excess of blasts/acute myeloid leukemia) in 4, 2, 13, and 0 cases, respectively. Nine pat...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Original Articles Source Type: research

Effect of Sickle Cell Anemia Therapies on the Natural History of Growth and Puberty Patterns
As pediatric patients with sickle cell anemia (SCA) have impaired growth and puberty patterns, we studied the effect of disease-modifying therapies on growth and puberty patterns for patients with SCA receiving hydroxyurea (HU), transfusions, or no therapy. We performed a retrospective study of children with SCA in whom anthropometric measurements and therapy type were recorded. Penalized smoothing splines were fitted to estimate growth curves and growth velocity, and linear mixed models were used to examine differences across treatment groups. Across group analyses were divided into early childhood (4.0 to 7.9 y) a...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Original Articles Source Type: research

How Does Comprehensive Care Impact Life of Pediatric Patients With Hemophilia? Results From a Center in a Developing Country
Background: Quality of life (QoL) has been included as a marker of treatment effectiveness in pediatric patients with chronic diseases. We believe that frequent multidisciplinary interventions and patient education could lead to an improvement in QoL. Aims: Determine the QoL and economic impact of monthly interventions in multidisciplinary treatment. Materials and Methods: The Haemo-QoL questionnaire was applied to patients who attended the hemophilia center of the University Hospital “Dr. José Eleuterio González,” Monterrey, Mexico, at the time of enrollment and 1 year later. Results: ...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Original Articles Source Type: research

Diagnosis of Invasive Fungal Infection Among Pediatric Oncology Patients
Discussion: On the basis of the inferior diagnostic yield of BAL, we believe that VATS-biopsy may be a more useful diagnostic adjuvant in the diagnosis of IFI in the immunocompromised pediatric oncologic patient population. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Original Articles Source Type: research

Neuroblastoma in Adolescents and Children Older than 10 Years: Unusual Clinicopathologic and Biologic Features
In conclusion, NB in children older than 10 years may exhibit unusual clinicopathologic and genetic features with large tumors, bilateral adrenal disease, rare morphologic features, complex DNA microarray findings and novel mutations. Patients often have grim prognoses despite genomic profiling-guided targeted therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Original Articles Source Type: research

Parental Attitudes Towards Prenatal Genetic Testing For Sickle Cell Disease
This study evaluated the association of knowledge and attitudes towards prenatal genetic counseling (PGC), awareness of posttesting intervention options and omission bias with attitudes towards PGT. In addition, we explored changes among knowledge, attitudes, and awareness of options following exposure to an educational, clinical vignette among parents of children with SCD. Parents (n=44) completed a questionnaire and an educational, clinical vignette presenting a detailed account of a pregnant woman with sickle cell trait seeking PGT and PGC was read to each participant. t Tests, Spearman correlations, multivariable regre...
Source: Journal of Pediatric Hematology Oncology - October 23, 2019 Category: Hematology Tags: Original Articles Source Type: research

Acute Lymphoblastic Leukemia With INPP5D-ABL1 Fusion Responds to Imatinib Treatment
We describe a patient with Down syndrome whose precursor B-cell acute lymphoblastic leukemia cells expressed INPP5D-ABL1 fusion gene that resulted in a reciprocal chromosome translocation t(2;9)(q27;q34). The fusion gene was present as a small subclone in the primary disease but was first identified at relapse when the subclone had expanded into a major clone. At relapse, the patient responded poorly to conventional induction chemotherapy but a transient morphologic remission was achieved after administration of imatinib monotherapy. This case demonstrates a pathway to relapse in a Down syndrome patients with acute lymphob...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Early Testing of Serum Ferritin Facilitates Hemophagocytic Lymphohistiocytosis Diagnosis in Children
We report the value of testing serum ferritin level early in the disease process in 3 pediatric patients who presented with persistent fever and sepsis-like features. Detection of elevated serum ferritin levels facilitated further testing to confirm the diagnosis of HLH and initiate early therapy with good outcomes. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Parenteral Nutrition Complications in Children Undergoing Bone Marrow Transplantation
Conclusions: Although we have noticed multiple complications attributable to PN, a certain causal link is difficult to establish in this particular context. Emphasizing enteral nutrition in bone marrow graft protocols would be the best way to avoid these complications. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Brincidofovir as a Salvage Therapy in Controlling Adenoviremia in Pediatric Recipients of Hematopoietic Stem Cell Transplant
We present a review of adenovirus infections treated with BCV which were unresponsive to cidofovir initially in 4 patients and it was used upfront in one patient. Children with adenovirus infection following HSCT treated with BCV, between July 2014 and February 2018 were recognized. Five patients including 3 male and 2 female with a median age of 10 years (range, 2.2 to 10 y) were identified. The median days of adenoviremia detection was 18 days (range, 7 to 303 d) posttransplant. The median peak viral load by quantitative polymerase chain reaction was 21,38,000 copies/mL (range, 1,77,200 to 31,97,000&...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Thiol Disulfide Homeostasis and Ischemia-modified Albumin Level in Children With Beta-Thalassemia
This study aimed to investigate the relationship of thiol levels, SH/SS homeostasis, and ischemia-modified albumin (IMA) in patients with β-thal. Materials and Methods: A hundred transfusion-dependent β-thal patients and 41 healthy controls were included in the study. Results: Native thiol, total thiol, disulfide, catalase, and IMA levels were significantly higher in the β-thal group compared with the control group (P (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Typical or Atypical Hemolytic Uremic Syndrome and the Use of Eculizumab: 4 Illustrative Cases
We reported here clinical cases where eculizumab use was probably not appropriate once the correct diagnosis of typical HUS was made and cases where it would have been useful because of the late diagnosis of aHUS. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Prevention of Long-term Adverse Health Outcomes With Cardiorespiratory Fitness and Physical Activity in Childhood Acute Lymphoblastic Leukemia Survivors
Conclusions: Our results indicate that a good cardiorespiratory fitness and physical activity level induced a preventive action for most health outcomes studied and was associated with a lower late adverse effects prevalence in ALL survivors. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Magnetic Resonance–guided High-intensity Focused Ultrasound (MRgHIFU) Virtual Treatment Planning for Abdominal Neuroblastoma Utilizing Retrospective Diagnostic 3D CT Images
This study sought to demonstrate that neuroblastomas are targetable with MRgHIFU and available computed tomographic imaging could be utilized for MRgHIFU virtual treatment. Cross-sectional images of 88 pediatric abdominal neuroblastoma patients were retrospectively processed with custom software to be made compatible with the Sonalleve MRgHIFU platform. Targetability measured percent treatment to lesion volume, within adequate safety margins from critical structures. All images were successfully converted into treatment planning files. Median lesion size was 191±195 cm3 and depth was 29±17 mm. U...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Longitudinal Trend in Emergency Department Reliance for Pain Among Sickle Cell Disease Patients in Wisconsin
This study uses Wisconsin Medicaid data from 2011 to 2015 to examine trend of EDR for pain over the period of 5 years. We stratified our cohort (N=750) by patient ages into 4 groups: (1) children; (2) transition group; (3) young adults; and (4) adults. Using a linear mixed model, we estimated longitudinal trends adjusting for age group and hydroxyurea possession calculated as medication possession ratio. Results show that EDR for pain has distinct temporal patterns for each group. EDR for pediatrics continually remained less than the established threshold of 0.33. The EDR for transition group significantly increased over t...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Central Venous Line Associated Deep Vein Thrombosis in Hospitalized Children
An increase in the incidence of deep vein thrombosis (DVT) has been reported in pediatric patients over the past decade. The presence of central venous line (CVL) is a major contributing risk factor with conflicting data on the relative risk of DVT with various types of central lines. We aimed to assess the incidence of and identify potential risk factors for DVT overall and with different types of CVL individually. A retrospective chart review of pediatric patients with a CVL placed at Cleveland Clinic Children’s from 2011 to 2016 was conducted. Data collected included demographics, potential risk factors, CVL chara...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Prognostic Factors and Treatment Outcome of Pediatric Anaplastic Large Cell Lymphoma Treated at the Children Cancer Hospital Egypt
Conclusion: Treatment of ALCL according to the Children’s Oncology Group ANHL 0131 protocol is well tolerated. The relapsing patient could be salvaged by high-dose chemotherapy and autologous hematopoietic stem cell transplantation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Providers’ Perspectives on Treating Patients With Thalassemia
In recent years, California has experienced a steady rise in Asian immigration which has led to a corresponding increased prevalence of clinically significant thalassemia in this state. As part of the Public Health Research, Education and Surveillance for Hemoglobinopathies emoglobinopathies project, a survey was developed to collect information from California providers who care for thalassemia patients in an effort to better understand their practice patterns, barriers to providing care, and educational needs. When asked about educational needs, providers most frequently expressed a desire for care and management guideli...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Hyperimmunoglobulinemia and IgG Subclass Abnormalities in Children With Gaucher Disease
Gaucher disease (GD) is the most common lysosomal storage disorder, the aim of the current study was to investigate hyperimmunoglobulinemia and abnormalities of serum immunoglobulin G (IgG) subclasses in children with GD and the relation of those findings to the GD phenotype and genotype, duration of enzyme replacement therapy (ERT), and infection frequency. The study included 20 Egyptian children with GD receiving ERT and 20 age-matched and sex-matched healthy children as controls. Serum Ig and serum IgG subclass levels were measured in the children with GD. Serum IgG subclass levels were measured in the control subjects....
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Cytokine Profile, Apoptosis, Glucocorticoid Receptor, and P-glycoprotein Expression Before and After Megadose Methylprednisolone Treatment in Children With Acute Immune Thrombocytopenia
Objective: Immune thrombocytopenia (ITP) is an autoimmune disease, and it has become evident that T lymphocytes play an important role in the pathogenesis of ITP. We investigated the role of T helper (Th) intracellular IL-2, IL-4, IL-6, IFN-γ, and T lymphocyte apoptosis in the pathogenesis of acute ITP and the effect of glucocorticoid treatment on cytokine profile. We investigated also P-glycoprotein (P-gp) and glucocorticoid receptor (GCR) expression as a possible mechanism for glucocorticoid resistance. Material and Methods: The study includes 20 children with acute ITP having a platelet count (Source: Journal...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Intraventricular Rituximab in Pediatric CD20-positive Refractory Primary Central Nervous System Lymphoma
Primary central nervous system lymphoma (PCNSL) is a rare and aggressive type of extranodal non-Hodgkin lymphoma that carries an unsatisfactory prognosis. Treating refractory PCNSL is challenging because of resistance to conventional cytotoxic and intrathecal chemotherapies. Therefore, novel therapeutic approaches are needed. Here, we report a 12-year-old boy with CD20-positive PCNSL, which was refractory to combination chemotherapy and intravenous rituximab. However, the patient achieved complete remission after repeated intraventricular rituximab administration. The results of this case indicate that intraventricular rit...
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Multifocal Appendiceal Carcinoid Tumor in an Adolescent: A Case Report and Review of the Literature
This report describes a case of a multifocal appendiceal carcinoid tumor identified incidentally following appendectomy in an adolescent. In this report, we describe the staging process and surgical management for focal and locally invasive appendiceal carcinoid tumors and highlight the rarity of multifocality in this location. The diagnostic and pathologic challenges for this case are presented. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Acceptability and Feasibility of a Disease-specific Patient Portal in Adolescents With Sickle Cell Disease
Conclusions: Electronic portals are promising tools for improving medical self-management. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Acute Swollen Calf Pain With a Diagnosis of Hemophilia Following an MRI
We report a young child who had a minor injury and calf swelling. He was initially diagnosed with deep vein thrombosis (DVT). Magnetic resonance imaging confirmed intramuscular hematoma and factor VIII assay confirmed hemophilia. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - October 1, 2019 Category: Hematology Tags: Radiology Corner Source Type: research