The Evolution of the Pediatric Hematology/Oncology Fellowship: What Does it Mean to be Productive?
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Letter to the Editor Source Type: research

Primary Pancreatic Neuroblastoma in an Infant
A 2-month-old girl with conjugated hyperbilirubinemia was found at the surgery and by computed tomography to have a large mass originating in the pancreas. Histopathology, molecular testing, and staging evaluations showed this to be a stage 3, MYCN unamplified, intermediate-risk neuroblastoma. The patient had a partial response to risk-stratified chemotherapy. The mass remained unresectable, but the response was sustained after 18 months. Although fewer than a dozen cases of primary pancreatic neuroblastoma have been reported, our experience and a literature review suggest that these tumors can be managed in the same way a...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Very Rare Congenital Dyserythropoietic Anemia Variant—Type IV in a Patient With a Novel Mutation in the KLF1 Gene: A Case Report and Review of the Literature
We report the nineth case with congenital dyserythropoietic anemia type IV, with a novel mutation that has not been reported before. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Newborn With Skin Lesions, Thrombocytopenia, and Gastrointestinal Bleeding
A term infant girl was admitted for evaluation of severe thrombocytopenia. She also had purpura-like skin lesions. A complete blood count showed a platelet count of 40×109/L (normal value: 150 to 400×109/L). She received random donor platelet transfusions and intravenous immunoglobulin therapy; however, thrombocytopenia persisted. She developed bloody stools on day 5 of life and hematemesis on day 9. Upper gastrointestinal endoscopy revealed multiple small, 2 to 5 mm, vascular lesions throughout the stomach body and proximal duodenum. Our multidisciplinary team will discuss an approach towards a term inf...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Evaluation of Bleeding Phenotype of Inherited Factor VII Deficiency in Children With a Bleeding Assessment Tool and Global Assays
Introduction: Inherited factor VII (FVII) deficiency is the most common of the rare bleeding disorders and shows a heterogenous distribution of bleeding phenotypes independent of factor activity level. The bleeding score (BS) evaluates the phenotype of patients with rare bleeding disorders. Thromboelastography (TEG) and thrombin generation assays (TGAs) are 2 methods to evaluate global hemostasis, and controversially both tests are useful for identifying different bleeding tendency phenotypes. The purpose of this study was to investigate the use of the BS and global assays (TEG and TGAs) to predict the bleeding phenotype...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Successful Use of Hematopoietic Stem Cell Transplantation for 2 Pediatric Cases of Glanzmann Thrombasthenia and Review of the Literature
Glanzmann thrombasthenia is a rare platelet disorder characterized by an abnormal integrin receptor on the surface of platelets that results in the failure of platelets to aggregate. Currently, curative therapy is allogeneic hematopoietic stem cell transplantation (HSCT). The authors report 2 patients with Glanzmann thrombasthenia who successfully underwent allogeneic HSCT from unrelated donors, including one using umbilical cord blood stem cells. Although both patients had evidence of engraftment, hematopoietic recovery, and normalization of platelet aggregation, they also experienced several post-transplant complications...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Recurrent Stroke in a Child With Atlantoaxial Instability Following Chiropractic Manipulation
We report a case of a child with a bow hunter’s stroke that was challenging to diagnose. This type of stroke happens when the vertebral artery is occluded at the atlantoaxial or subaxial level during neck rotation. This case demonstrates that workup of stroke should be comprehensive to include all mechanical and anatomic possibilities before investigating rarer hypercoagulable disorders. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

What Do We Know About the Do-It-Yourself Diets of Patients in Our Care?
This study underscores the fact that many patients and families chose to impose dietary changes that are not always discussed with their health care staff and that may risk interfering with the continuity of a patient’s treatment. It is essential to have more opportunities to speak with patients and families about this issue. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Development and Validation of the Warfarin-Aspirin Bleeding Assessment Tool (WA-BAT) in Children
Bleeding assessment tools (BATs) aim to screen and estimate bleeding risk in patients with inherited bleeding disorders. However, the use of BAT as a standardized measure for comparing bleeding in patients on long-term thromboprophylaxis has not yet been validated. We developed a self-administrable BAT to assess bleeding in patients undergoing long-term thromboprophylaxis with aspirin or warfarin. Eligible participants were invited to complete the warfarin-aspirin -BAT (WA-BAT) online. The WA-BAT was readministered a number of weeks later to determine intrarater reliability. The WA-BAT showed substantial intrarater reliabi...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Severe Factor X Deficiency Presenting as Febrile Seizure in an Infant
We present the case of an infant with factor X deficiency who presented with complex febrile seizure. Although febrile seizures are very common in children, a closer scrutiny leads to neuroimaging and finding of intracranial bleed. Hematologic and genetic investigations confirmed the diagnosis. A high index of suspicion should be maintained to diagnose uncommon bleeding disorders in children. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Hematopoietic Stem Cell Transplantation in Congenital Dyserythropetic Anemia Type II: A Case Report and Review of the Literature
Currently, there is no guideline for the treatment of patients with congenital dyserythropoietic anemia (CDA) type II. One approach is to follow-up patients with transfusions, on the basis of individually determined target hemoglobin levels, and iron chelation according to the thalassemia guidelines. In some transfusion-dependent CDA II patients, splenectomy reduces the number of transfusions; however, the only known curative option for CDA II patients is hematopoietic stem cell transplantation (HSCT). Only a few published case reports of allogeneic HSCT in CDA II patients are available. Here, we review the literature and ...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Pulmonary Artery Pseudoaneurysm in a Child With β-Thalassemia Major
Pulmonary artery aneurysms and pseudoaneurysms are rare vascular anomalies in children that can lead to massive hemoptysis resulting in severe morbidity and even mortality. High level of clinical suspicion, timely diagnosis, and prompt management are important for a better outcome. Here, we report a case of a 14-year-old adolescent with β-thalassemia major who presented with life-threatening hemoptysis due to pulmonary artery pseudoaneurysm and was successfully treated with coil embolization. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Homozygous pArg610del Mutation Unusually Associated With Severe Delay of Growth in 2 Acid Sphingomyelinase Deficiency–affected Sibs
Conclusions: There is no correlation between phenotype-genotype in case of p.Arg610del mutation that could be associated to a severe delay of growth. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Klebsiella pneumoniae Multiple Liver Abscesses and Bacteremia in a Transfusion-dependent β Thalassemia Major Patient
We report a 16-year-old splenectomized transfusion-dependent thalassemia major patient who presented with multiple liver abscesses with KP bacteremia. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Non–transfusion-dependent β-Thalassemia Because of a Single β-Thalassemia Mutation and Coinherited α-Globin Gene Triplication: Need for Increased Awareness to Prevent Incorrect and Delayed Diagnosis
We describe 5 unrelated individuals with non–transfusion-dependent β-thalassemia (NTDT), some with apparently dominant transmission, because of a single β-thalassemia mutation coinherited with a triplicated α-globin locus. Each had an initial, incorrect diagnosis of β-thalassemia trait. The correct diagnosis of NTDT was made at a mean of 7 years of age. Despite reports of this compound genotype causing NTDT, it remains unfamiliar to many clinicians. To increase awareness, we highlight its varied and sometimes subtle clinical and laboratory features and the need for comprehensive genetic testing f...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Case of Langerhans Cell Histiocytosis With Multifocal, Single-System GI Tract Involvement and Literature Review
We describe a 20-year-old Hispanic female with multifocal, single-system gastrointestinal LCH. Initially diagnosed from a CD1a, S100, and CD207 (Langerin) positive appendix tissue after an appendectomy and confirmed multifocal with an endoscopy. She had a full clinical and endoscopic resolution of disease with cytarabine therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Late Recurrence of Wilms Tumor After 17 Years: A Case Report
We describe a case of late recurrence of Wilms tumor as a remote metastasis in the lung at 18 years after the first diagnosis and 17 years after the second remission, which was achieved by radiotherapy and high-dose chemotherapy with autologous stem cell rescue. After late recurrence, the patient was treated by surgery and adjuvant chemotherapy, and remained disease-free for 11 months. Several very late recurrences of Wilms tumor in the literature are reviewed. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Review of Infants With Localized Neuroblastoma That Evolve to Stage 4s Disease
The authors describe a newborn diagnosed with localized neuroblastoma that evolved to stage 4s at the age of 5 months. Peculiar features of the case included a bilateral adrenal primary, the skin as the only metastatic site, and the development of a muscular lesion late in the clinical course. The patient underwent left adrenalectomy and all other lesions regressed without further therapy. The case prompted a search for similar cases both in the Italian Neuroblastoma Registry and in the literature. All patients identified, although variously treated, survived with the exception of the 2 with MYCN gene amplification. We con...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Awareness of Hemophagocytic Lymphohistiocytosis as an Unusual Cause of Liver Failure in the Neonatal Period
Conclusion: Growing awareness of HLH as a cause of liver failure in the neonatal period can be associated with early treatment and reduces mortality in this group of patients. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Chemotherapy Wait Times in a Network of Pediatric Oncology Clinics
In conclusion, implementation of quality improvement interventions across a clinical network can improve specific aspects of patient satisfaction, thereby improving the overall patient experience. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Successful Treatment of an EBV-positive Diffuse Large B-Cell Lymphoma in a Patient With Trisomy 21
We describe the first case of EBV-positive DLBCL in a patient with trisomy 21 and we propose a treatment modality for this rare entity. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Isavuconazole Treatment of Cerebral and Pulmonary Aspergillosis in a Pediatric Patient With Acute Lymphoblastic Leukemia: Case Report and Review of Literature
Invasive aspergillosis in hematologic pediatric patients is an opportunistic infection that is difficult to treat, with a high mortality rate when localized in the central nervous system. We are describing a 3-year-old girl who was affected by acute lymphoblastic leukemia who developed cerebral and pulmonary aspergillosis during induction chemotherapy. The patient failed first-line voriconazole treatment because of being a CYP2C19 ultrarapid metabolizer and received effective isavuconazole therapy with no notable side effects. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Emergence of a Ph-negative Clone in a Child With Ph+ ALL
We describe an 8-year-old boy with Ph+ ALL relapsing with ALL without the Ph following treatment with dasatinib as a part of Children’s Oncology Group trial AALL1122. This emphasizes the polyclonal nature of ALL at diagnosis and indicates that the BCR-ABL fusion oncogene is not always an essential “driver” mutation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Hepatosplenic αβ T-Cell Lymphoma as Second Malignancy in Young Adult Patient With Previously Undiagnosed Ataxia-Telangiectasia
We present a case of a female individual diagnosed with T-cell acute lymphocytic leukemia at 13 years and subsequently with αβ subtype of hepatosplenic T-cell lymphoma (HSTCL) at 20 years. During her diagnostic work up for HSTCL, paired tumor-germline sequencing identified a diagnosis of ataxia-telangiectasia. We also describe a very refractory clinical course of her αβ HSTCL, including only a brief response to multiagent chemotherapy and an allogenic bone marrow transplant. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Case of Childhood Blastic Phase Chronic Myeloid Leukemia With Minor BCR-ABL
We present a child with blastic phase CML associated with minor BCR-ABL transcript without prior CML diagnosis. Diagnosis was achieved by fluorescence in situ hybridization of peripheral blood neutrophils, which identified 90% as BCR-ABL positive. The patient received chemotherapy with imatinib followed by dasatinib and underwent reduced-intensity hematopoietic allogeneic stem cell transplantation with prophylactic posttransplant dasatinib for 2 years and has remained in complete molecular remission. Our intensified treatment regimen was effective compared with previous studies on minor BCR-ABL CML describing inferior outc...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Brentuximab Vedotin and High-dose Methotrexate Administrated Alternately for Refractory Anaplastic Large-cell Lymphoma With Central Nervous System Disease
Pediatric anaplastic large-cell lymphoma (ALCL), which is characterized by strong expression of CD30, is usually responsive to multidrug chemotherapy. Brentuximab vedotin (BV) which is an anti-CD30 antibody-drug conjugate is a promising drug with effects on relapsing or refractory ALCL. However, its effects may not be sufficient for the central nervous system disease. The authors herein reported an 11-year-old boy with ALCL that progressed as central nervous system disease receiving intensive induction chemotherapy has achieved and maintained remission by BV and high-dose methotrexate administrated alternately. Alternate t...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Survival and Neurologic Recovery After Prompt Diagnosis and Aggressive Management of Severe Idiopathic Hyperammonemic Encephalopathy in a Patient with Acute Myeloid Leukemia
A case of a 19-year-old female with low-risk acute myeloid leukemia is presented who was diagnosed with idiopathic hyperammonemic encephalopathy following the development of abrupt neurologic decline, respiratory alkalosis, and elevated plasma ammonia levels of unknown etiology. Delayed symptom recognition of this exceedingly rare condition contributes to the often fatal outcomes of idiopathic hyperammonemic encephalopathy. As illustrated by this case, prompt diagnosis and utilization of a variety of ammonia-modulating treatment modalities can result in remarkable clinical recovery. This case provides guidance to clinician...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Pediatric Febrile Neutropenia: Change in Etiology of Bacteremia, Empiric Choice of Therapy and Clinical Outcomes
Background: The optimal choice of initial antibiotic therapy for patients with high-risk febrile neutropenia (FN) in children is unclear and varies by the institution on the basis of local antibiograms and epidemiology of specific pathogens. The authors evaluated the appropriateness of antibiotics for the empiric treatment of FN in pediatric patients with cancer in our institution on the basis of changes in the epidemiology of organisms isolated from blood cultures (BCx). Methods: The authors conducted a retrospective medical record review of pediatric patients who received any oncology care (including patients with c...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Hemophagocytic Lymphohistiocytosis and Graft Failure Following Unrelated Umbilical Cord Blood Transplantation in Children
Hemophagocytic lymphohistiocytosis (HLH) following hematopoietic stem cell transplantation is closely correlated with graft failure and poor prognosis. Because of its rarity, the incidence, risk factors, and optimal treatment strategy are unclear. We analyzed data from cases of HLH following umbilical cord blood transplantation (UCBT) performed for pediatric patients at our center. Among 66 UCBT recipients, 5 developed HLH and imminent graft failure. The median time of diagnosis of HLH was 22 (range, 19 to 30) days after UCBT, and the cumulative incidence of HLH was 7.6% (95% confidence interval, 2.8-15.7) at day 60. In un...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Hemophagocytic Lymphohistiocytosis in Patients With Primary Immunodeficiency
We presented a single-center experience consisting of patients with HLH secondary to PID with a mortality rate of 32.1%. Hypoalbuminemia was the only risk factor to increase the overall mortality rate of HLH. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Isolated Central Nervous System Relapse Following Treatment Reduction in Low-risk Acute Lymphoblastic Leukemia at the Children’s Cancer Center of Lebanon
The aim of this trial was to decrease the incidence of life-threatening infections by decreasing the dose and the duration of dexamethasone treatment during maintenance therapy. This was a prospective, nonrandomized trial of low-risk acute lymphoblastic leukemia patients 1 to 18 years of age who were treated at the Children’s Cancer Center of Lebanon (CCCL). Patients consecutively diagnosed between 2002 and 2013 were divided into groups 1 and 2 receiving total dexamethasone doses of 1144 and 618 mg/m2, respectively. A total of 84 patients were assigned to group 1 and 33 patients to group 2. The 5-year cumulati...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Outcome of Core Binding Factor Acute Myeloid Leukemia in Children: A Single-Center Experience
Childhood acute myeloid leukemia (AML) harboring core binding factor (CBF)–associated translocations are considered as a favorable cytogenetic subgroup. The 2 major subtypes of CBF-AML include t(8;21) and inversion of chromosome 16, accounting for ∼25% of patients. Because of expensive and toxic treatment, which may require hospitalization during the entire course of induction chemotherapy, most of the centers in Pakistan neither workup for this low-risk entity nor offer curative treatment. Therefore, we adopted an approach of screening AML cases for the presence of CBF with the rationale of offering curative tre...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Procalcitonin Clearance, CD64, and HLA-DR as Predictors of Outcome in Febrile Neutropenic Children With Lymphoreticular Malignancies
Conclusions: On the basis of the findings of the study we suggest that serial monitoring of procalcitonin levels be used in febrile neutropenic children with cancer. Procalcitonin levels on day 3 alone can be offered in resource poor setting. The role of HLA-DR and CD64 also seems promising and needs to be further explored in larger multicentric studies. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Characteristics of Blood Transfusion During Induction Remission in Children With Acute Lymphoblastic Leukemia: A Single-Center Retrospective Investigation
Objective: To investigate the allogeneic blood transfusion (ABT) characteristics of children with acute lymphoblastic leukemia (ALL) in different risk stratification during vincristine, daunorubicin, L-asparaginase and prednisone (VDLP) induction remission. Subjects and Methods: By referring to electronic medical records, the demographic characteristics, diagnosis, test, and treatment information including ABT were collected. According to the risk stratification of the CCCG-ALL-2015 protocol, ABTs between groups were compared, and the differences were statistically analyzed. Results: One hundred sixty-three newly t...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Venous Thromboembolism After Peripherally Inserted Central Catheters Placement in Children With Acute Leukemia: A Single-center Retrospective Cohort Study
Conclusions: The incidence rates of symptomatic VTE after PICC placement in children with AL in our center were relatively low and acceptable. For patients with AML, PICC placement plays the most important role in venous thrombosis. However, in patients with ALL, the potential risk factors were more complex. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

IKZF1 Deletions as a Prognostic Factor in Costa Rican Patients With Pediatric B-Cell Acute Lymphoblastic Leukemia
The IKZF1 gene encodes for Ikaros, a transcriptional factor in B-cell development. Deletions in this gene have been associated with a worse prognosis in B-cell acute lymphoblastic leukemia (B-ALL). We evaluated the presence of these alterations in all Costa Rican pediatric patients diagnosed with B-ALL between 2011 and 2014, treated with a modified Berlin-Frankfurt-Münster therapeutic protocol. Multiplex polymerase chain reaction with 2 detection methods (agarose gel and gene scanning) was used to detect intragenic deletions and multiplex ligation-dependent probe amplification for whole-gene deletions. Differences bet...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Retinal Vessel Analysis as a Novel Screening Tool to Identify Childhood Acute Lymphoblastic Leukemia Survivors at Risk of Cardiovascular Disease
Background: Microvascular endothelial dysfunction is central to the pathogenesis of cardiovascular disease (CVD). The eye offers direct access for endothelial health assessment via the retinal microvasculature. The aim of the study was to investigate whether image-based retinal vessel analysis is a feasible method of assessing endothelial health in survivors of childhood acute lymphoblastic leukemia (cALL). Materials and Methods: Cardiovascular risk factors (CRFs) were estimated using the 30-year Framingham Risk Score in 73 childhood leukemia survivors (median age: 25; median years from diagnosis: 19) and 78 healthy c...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

COVID-19 in Children With Blood and Cancer Disorders: What Do We Know So Far?
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Letters to the Editor Source Type: research

Teaching Versus Nonteaching Hospitals: Where Children With Sickle Cell Disease Receive Their Emergency Department Care
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Letters to the Editor Source Type: research

Fusobacterium nucleatum Multiple Liver Abscesses in an Adolescent With Hemoglobin SC Disease
We report here multiple liver abscesses occurring in a 17-year-old patient with hemoglobin SC disease. A Fusobacterium nucleatum was identified on cyst puncture. Such complications have been described in only 11 children and young adults with hemoglobin SS/Sβ0-thalassemia diseases. Fusobacterium species are the most frequent pathogens reported and require anaerobic culture to be identified. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Radiology Corner Source Type: research

Irritability and Perceived Expressed Emotion in Adolescents With Iron Deficiency and Iron Deficiency Anemia: A Case-Control Study
The objective of this study was to assess the difference in irritability and perceived expressed emotion (EE) between adolescents with iron deficiency (ID) or iron deficiency anemia (IDA) and their healthy peers. In addition, we aimed to investigate the relationship between hemogram parameters, irritability, and perceived EE in adolescents with ID and IDA. The sample of this single-center cross-sectional case-control study consisted of 89 adolescents from 12 to 17 years of age. Of the participants, 19 had been diagnosed with ID, 31 had IDA, and 39 were healthy controls. Significant differences in the self-reported and pare...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Original Articles Source Type: research

Cardiac Iron Overload by MRI in Children With B-Thalassemia Major and its Correlation With Cardiac Function by Echocardiography
Background: Serial echocardiography is strongly recommended in asymptomatic B-thalassemia major (TM) patients for early detection of subtle cardiac dysfunction. T2*magnetic resonance imaging (MRI) is a noninvasive measurement of myocardial iron burden. Yet, it is not always available in many centers. Our study aimed to evaluate the myocardial function in TM patients using different echocardiographic modalities and to correlate these findings with cardiac T2*MRI. Patients and Methods: This is a cross-sectional study that was carried out on 140 children with a mean age of 10.9±3.7 years. One hundred children with...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Original Articles Source Type: research

Tilt-table Echocardiography Unmasks Early Diastolic Dysfunction in Patients With Hemoglobinopathies
This study sought to determine whether changes in left atrial (LA) function during manipulation of cardiac preload by tilt-table echocardiography can unmask subclinical diastolic dysfunction in pediatric patients with hemoglobinopathies. Eleven sickle cell anemia, 9 transfusion-dependent thalassemia major, and 10 control subjects underwent tilt-table echocardiogram in the supine (loading) and 30-degree upright (unloading) positions and cardiac magnetic resonance imaging (MRI). Echocardiography assessed LA and left ventricular (LV) strain, strain rate, mitral inflow, and annular velocities. MRI assessed LV function, myocard...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Original Articles Source Type: research

The Effects of L-Carnitine on Echocardiographic Changes in Patients With β-Thalassemia Major and Intermedia
Introduction: Heart failure, fatal arrhythmias, and cardiac dilatation because of anemia are common causes of β-thalassemia major–related deaths. The aim of this study was to determine the effect of L-carnitine on echocardiographic changes in β-thalassemia major and intermedia patients in Besat Hospital in Sanandaj, Iran. Methods: In a randomized clinical trial, 60 β-thalassemia patients who were eligible for L-carnitine administration were randomly divided into 2 placebo and study drug groups. The duration of the study was 6 months. Using echocardiography and blood tests, cardiac parameters inclu...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Original Articles Source Type: research

An Evaluation of Bone Health Parameters in Regularly Transfused Beta-Thalassemia Major Patients
As beta-thalassemia major patients need regular blood transfusions due to the severe hemoglobin deficiency, the occurrence of related bone defects with simultaneous fluctuations in the biochemical and hematologic parameters is seen. The hospital-based cross-sectional observational study was done to determine and correlate the bone mineral density (BMD) with biochemical parameters and hematologic parameters in 50 regularly transfused beta-thalassemia major patients of older than 6 years of age. Descriptive statistics were analyzed with SPSS version 20.0 software. A P (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Original Articles Source Type: research

Emergency Department Provider Survey Regarding Acute Sickle Cell Pain Management
Sickle cell disease (SCD) can lead to potentially devastating complications that occur secondary to vaso-occlusion. Current national clinical guidelines are largely based on expert opinion, resulting in significant variation of management. Provider awareness regarding emergency department (ED) management of vaso-occlusive crises (VOC) remains unknown. A 23-question assessment of VOC management was administered to all eligible ED providers at Riley Hospital for Children between September and November 2018. Univariate analyses were performed to evaluate responses between groups. Of 52 respondents comprised of ED staff attend...
Source: Journal of Pediatric Hematology Oncology - July 30, 2020 Category: Hematology Tags: Original Articles Source Type: research

A Unique Case of a Pediatric Patient With Blastic Plasmacytoid Dendritic Cell Neoplasm, Guillain Barre Syndrome, and Hemophagocytic Lymphohistiocytosis
We describe a pediatric patient with blastic plasmacytoid dendritic cell neoplasm who subsequently developed Guillain Barre syndrome followed by hemophagocytic lymphohistiocytosis. All 3 conditions are uncommon, particularly in the pediatric population. It is unclear whether this patient developed these disease states independently, whether they were due to a viral trigger or if she has an underlying immune dysfunction that could have contributed to the development of these conditions. The patient is currently in remission and awaiting further immune work-up. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 25, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Benign Phyllodes Tumor in Children: A Study of 8 Cases and Review of the Literature
Phyllodes tumor (PT) occurs predominantly in middle-aged women, and although its occurrence in young women, adolescents, and even children is documented, presentation in the pediatric population has been the least well studied because of its rarity. Incompletely defined in children with PT are recurrence rates and optimal surgical management. We retrospectively studied the pathology database of Hartford Hospital from 2010 to 2017 to find all cases of PT in patients 18 years of age or younger. A series of 8 children/adolescents with breast masses diagnosed as benign PT were identified. Patients were 14 to 16 years of age (m...
Source: Journal of Pediatric Hematology Oncology - June 25, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Roseomonas gilardii Bacteremia in a Patient With HbSβ0-thalassemia: Clinical Implications and Literature Review
Roseomonas gilardii is a Gram-negative coccobacillus identified in immunocompromised pediatric patients. A 5-year-old male with a history of HbSβ0 thalassemia status postsurgical splenectomy presented to the emergency department with fever. Blood cultures grew R. gilardii at 63 hours, but the patient had been discharged home at 48 hours. The patient was readmitted for repeat cultures and initiated on meropenem for 10 days as Roseomonas spp. are often resistant to third generation cephalosporins. R. gilardii is a rare cause of bacteremia in immunocompromised patients. Clinicians should consider Roseomonas in slow growi...
Source: Journal of Pediatric Hematology Oncology - June 25, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Challenges in the Diagnosis of Medulloblastoma Recurrence at an Unusual Site in a Patient With Prader-Willi Syndrome
We describe a 10.5-year-old girl with PWS and previous standard-risk medulloblastoma that relapsed in the pons 3 years after the end of treatment. Diagnosis of relapse was delayed by a preceding varicella infection, an initial clinical/radiologic response to steroids and the unusual location, and was confirmed with a stereotactic biopsy. Second-line therapy was commenced, however, the patient rapidly deteriorated and died. This is the first report of medulloblastoma in a patient with PWS. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 25, 2020 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research