May-Thurner Syndrome: An Underdiagnosed Entity
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

Favorable Response to Interferon-α in Infantile-onset Idiopathic Hypereosinophilic Syndrome Complicated by Status Epilepticus During Treatment
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

Pediatric Chronic Myeloid Leukemia Presenting With Extreme Thrombocytosis and Acute Upper Gastrointestinal Hemorrhage: A Case Report
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

Pulmonary Toxicity From Granulocyte Colony Stimulating Factor in a Pediatric Patient Undergoing Autologous Stem Cell Harvest
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

COVID-19 in Children and Adolescents With Cancer From a Single Center in Mexico City
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

Successful Implementation of Routine SARS-CoV-2 Screening in Children With Cancer and Their Parents During the Pandemic in the United Kingdom
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

Favorable Outcome of COVID-19 Infection in a Pediatric Cancer Patient Receiving an Anti-PD-L1/Anti-CTLA-4 Combination
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Letters to the Editor Source Type: research

Management of Anticoagulation Therapy in Patients With Thromboembolism in the Context of Renal Dysfunction: Challenging Cases and Practical Algorithms
Conclusions: A significant change in anti-factor Xa (anti-Xa) levels—used for drug monitoring—should prompt an assessment of renal function. In nephrotic syndrome, anti-Xa levels should be closely monitored when there is a change in the status of nephrotic disease activity. In hemodialysis patients, enoxaparin at once daily reduced dosing should be considered with trough and peak anti-Xa levels monitoring. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Infantile Pyknocytosis in a Premature Dichorionic Diamniotic Twin
We present a case of a premature twin, in whom genetic screening was performed. Genetic mutations in 46 genes associated with hereditary hemolytic anemia and dyserythropoietic anemia were tested. No mutations were found. In infantile pyknocytosis, a genetic defect in these genes is unlikely. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Haploidentical Stem Cell Transplantation With Post-transplant Cyclophosphamide for Pediatric Acute Leukemia is Safe and Effective
Conclusion: Haploidentical HSCT with PTCy is a safe and effective therapy for children with acute leukemia. Myeloablative conditioning and chronic GVHD lead to improved disease-free survival. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Haploidentical Stem Cell Transplant With Post-transplant Cyclophosphamide for Chediak-Higashi Syndrome: A Very Rare Case Report
Chediak-Higashi syndrome is a rare immunodeficiency disorder for which hematopoietic stem cell transplant (HSCT) is the only curative treatment option. HSCT only corrects the hematologic and immunologic manifestations of the disease but neurologic complications may still progress after transplant. Haploidentical HSCT (haplo-HSCT) has evolved as a feasible alternative for patients with primary immunodeficiency. More recently, there has been use of haplo-HSCT with post-transplant cyclophosphamide. However, only 4 cases of Chediak-Higashi syndrome have been reported using this approach. Here, the authors describe a case of a ...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Hematopoietic Stem Cell Transplantation for Hepatitis-associated Aplastic Anemia Following Liver Transplantation for Nonviral Hepatitis: A Retrospective Analysis and a Review of the Literature by the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation
Hepatitis-associated aplastic anemia (HAAA) has been reported in 23% to 33% of patients who received orthotopic liver transplantation (LT) for acute liver disease of unknown origin (nonviral hepatitis). In this situation, hematopoietic stem cell transplantation (HSCT) might be a curative option. Here the authors report on 6 patients who received HSCT after LT for nonviral HAAA hepatitis. The outcomes were interpreted in the context of recently reported immune suppressive therapy (IST) outcomes in 8 patients with HAAA and to HSCT outcomes in patients with HAAA who recovered from hepatitis without undergoing LT. All patients...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Comparable Outcomes of Matched Sibling Donor and Matched Unrelated Donor Stem Cell Transplantation in Children With Acute Leukemia in Argentina
Conclusions: In Argentina, we did not show significant differences in outcomes between MSD and MUD HSCT for children with high-risk leukemia. Future work should focus on strategies to reduce the relapse risk in children with high-risk leukemia in upper-middle-income countries. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Stage IV Classical Hodgkin Lymphoma-type Posttransplant Lymphoproliferative Disorder in a Pediatric Liver Transplant Patient: A Case Report and Review of the Literature
We describe a successfully diagnosed and treated CHL-PTLD stage IV pediatric patient, 8 years after liver transplantation. The patient was treated with standard CHL (Children’s Cancer Group 5942 group 3) chemotherapy, rituximab and reduction of immunosuppressant. The patient remains in complete remission after 3 years with stable graft function. To our best knowledge, this is the first pediatric case report of a successfully treated stage IV CHL-PTLD after a liver transplant. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Evaluation of Empiric Vancomycin for Fevers During High-dose Cytarabine Administration
Conclusion: Fever due to HDAC is relatively common but appears to frequently lack association with bacteremia during the time of HDAC administration. Broad-spectrum empiric antibiotic regimens including vancomycin may be unnecessary for these patients, particularly before they become neutropenic. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

A Retrospective Review of Mercaptopurine Metabolism Reveals High Rate of Patients With Suboptimal Metabolites Successfully Corrected With Allopurinol
Skewed drug metabolism of 6-mercaptopurine (6-MP) can jeopardize antileukemic effects and result in toxicities during the treatment of acute lymphoblastic leukemia and lymphoblastic lymphoma. Allopurinol can alter 6-MP metabolism to maximize therapeutic effects while reducing toxicities. Over 75% of our patients with acute lymphoblastic leukemia or lymphoblastic lymphoma experienced a 6-MP-related toxicity. Review of metabolite date a showed 6-methylmercaptopurine nucleotide levels were>10,000 in 55% of the cohort, suggesting 6-MP shunting. Allopurinol was initiated in 12 of 23 shunters with resolution of toxicities. We...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Incorporating Absolute Phagocyte Count With Absolute Neutrophil Count as a Measure for Safe Discharge for Pediatric Oncology Febrile Neutropenia: A Pilot Study
Adequate bone marrow recovery is a discharge requirement after admission for febrile neutropenia in oncology patients, without specific threshold in consensus guidelines. In January 2016, our institution implemented count recovery criteria of absolute neutrophil count ≥100 cells/μL and absolute phagocyte count ≥300 cells/μL compared with prior criteria of absolute neutrophil count ≥500 cells/μL. Retrospective analysis comparing pre (July 2013 to December 2015, N=68) and post (January 2016 to June 2018, N=30) groups showed no difference in readmissions (P>0.9), no patient deaths, and decreased average l...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Severe Vincristine-related Neurotoxicity in 5 Patients With Pediatric Acute Lymphoblastic Leukemia Requiring Discontinuation of Vincristine: A Description of Long-term Outcome
We report the clinical courses of 5 patients who required cessation of vincristine after developing severe neurotoxicity during treatment for acute lymphoblastic leukemia. All 5 patients lost the ability to ambulate and 3 had additional severe neurotoxic side effects including vision loss and vocal cord dysfunction. Although prior literature reports poor outcomes for children in whom vincristine was discontinued during acute lymphoblastic leukemia therapy, all 5 patients described here achieved and have maintained complete continuous remission. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Venetoclax and Decitabine in Pediatric Refractory T-cell Lymphoblastic Lymphoma
Conclusion: This case highlights the potential clinical activity of venetoclax and decitabine in relapsed T-LL. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Adjuvant Maintenance Larotrectinib Therapy in 2 Children With NTRK Fusion-positive High-grade Cancers
Treatment-related morbidity drives research to identify targetable lesions in children with cancer. Neurotrophic tropomyosin receptor kinase (NTRK) alterations occur in ~1% of pediatric solid tumors. Early phase pediatric trials involving the NTRK inhibitor treatment for progressive NTRK-mutated cancers show promising results. The authors describe the adjuvant maintenance larotrectinib treatment after definitive surgical resection in 2 toddlers with NTRK fusion-positive malignancies (ETV6-NTRK3 fusion-positive undifferentiated embryonal sarcoma of the kidney and NACC2-NTRK2 fusion-positive anaplastic astrocytoma). Both are...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Primary Primitive Neuroectodermal Tumor of the Spine With t(11;22): Report of 3 Cases and Review of Literature
Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. The authors also compare the clinical course and outcome of therapy with primary PNET of the central nervous system and Ewing sarcoma family of tumors. In addition, this report highlights the risk for l...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Multiple Intraspinal Gangliogliomas in a Child With Neurofibromatosis Type 1: Case Report and Literature Review
This report describes a 4-year-old girl with NF1 who was found to have multiple discrete, infiltrative intramedullary cord masses, and biopsy demonstrated World Health Organization grade I ganglioglioma. Panel-based next-generation sequencing showed her previously identified germline NF1 mutation and a second somatic NF1 mutation. This represents the first report of multiple primary intramedullary gangliogliomas in a child with NF1 and demonstrates how biopsy with panel-based next-generation sequencing provides potential targets for MAPK/MEK/BRAF pathway inhibitor therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Coronavirus Disease 2019 Infection in Children With Sickle Cell Disease: Case Series From Oman
Conclusion: Although SCD patients are considered as a high-risk group, all of the observed patients, and whose cases are reported here, have recovered. A large scale of SCD cases should be studied to reach more conclusive results. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Care Coordination in a SARS-CoV-2-infected Child With Newly Diagnosed Medulloblastoma and Fanconi Anemia
COVID-19, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is responsible for a global pandemic that can cause severe infections in children, especially those with comorbid conditions. Here, we report a case of a child with a newly diagnosed medulloblastoma, Fanconi Anemia, and SARS-CoV-2 infection. Through multidisciplinary care coordination and meticulous planning, we were able to safely initiate this patient’s oncology care and implement a long-term model to address the patient’s care. This approach could be replicated with any newly diagnosed pediatric patient that requires monitoring...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Febrile Neutropenia Syndromes in Children: Risk Factors and Outcomes of Primary, Prolonged, and Recurrent Fever
Background: The approach to recurrent febrile neutropenia (FN) in children with cancer has not been sufficiently addressed and was cited as a research gap in the International Pediatric Fever and Neutropenia (IPFNP) Guideline 2017. Methods: Retrospective medical record review for all pediatric cancer patients with a diagnosis of FN was performed. Variables were collected at 2 different time sets (at day 1 and day 4 of presentation). Three FN syndromes have been defined based on the duration and time course of the fever: (1) primary: fever resolved before 96 hours and did not follow with recurrent fever; (2) prol...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Epstein-Barr Virus-associated Pulmonary Tumor: A Pediatric Case and Discussion of the Literature
Epstein-Barr virus-associated smooth pulmonary tumor is a rare condition that mostly affects immunosuppressed patients. This case describes a young boy with a history of kidney transplantation who presented recurrent pneumonia. Multiple endobronchial soft tissue tumors affecting both right and left bronchial tree were found and partially removed by bronchoscopy. Immunohistologic analysis demonstrated Epstein-Barr virus-associated smooth pulmonary tumor. Immunosuppressive therapy was changed from tacrolimus to sirolimus. A few months later, new right upper lobe and inferior left lobe tumors were found. Recurrent left lower ...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Altered von Willebrand Factor and ADAMTS13 Levels in Children With Cirrhosis and Extrahepatic Portal Hypertension
This study was concerned with whether vWF (von Willebrand factor) and a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13 (ADAMTS13) has altered in patients with cirrhosis and extrahepatic portal hypertension (EPH). We aimed to investigate changes to vWF and ADAMTS13 in children with cirrhosis and EPH. Patients and Methods: This study was conducted between January and October 2019 with both cirrhosis and EPH patients and with healthy volunteers. The von Willebrand factor antigen (vWF:Ag), von Willebrand Ristocetin cofactor (vWF:RCo), and ADAMTS13 antigen and activity were studied. Resu...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Enoxaparin Dose Requirements to Achieve Therapeutic Low–molecular-weight Heparin Anti-factor Xa Levels in Infants and Young Children
Introduction: Enoxaparin is commonly used to treat pediatric thrombosis. Several small retrospective studies have suggested that infants and young children require higher enoxaparin doses to achieve therapeutic anti-factor Xa levels compared with adults. Materials and Methods: This is a retrospective study of hospitalized children who received enoxaparin for the treatment of thrombosis at a free-standing children’s hospital. The primary objective was to ascertain the enoxaparin dose required to achieve an anti-factor Xa level of 0.5 to 1.0 U/mL among 4 age groups in a large cohort of infants and you...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Association of Myeloperoxidase Gene Polymorphism With Iron Deficiency Anemia in Turkish Children
This study was performed to investigate the gene polymorphisms of the myeloperoxidase (MPO) enzyme and to determine whether MPO gene polymorphisms influence the response to iron therapy in pediatric patients with iron deficiency anemia (IDA). In this case-control study, 50 Turkish children with IDA and 50 healthy controls were enrolled. Three MPO gene alleles were selected for genotyping in the study: GG, AG, and AA. The relationships of alleles with IDA were analyzed and compared in patients and controls. Pretreatment and posttreatment laboratory parameters and gene polymorphisms were compared in the patient group. There ...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Is Monitoring of Cytomegalovirus Disease Required in Nontransplant Pediatric Acute Lymphoblastic Leukemia?
Conclusion: This study suggests the importance of monitoring for CMV infections in developing countries because of frequent reactivations in seropositive ALL patients. It should be kept in mind that low CMV-DNA levels may also lead to organ involvement. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Evaluation of Risk Factors Causing Ototoxicity in Childhood Cancers Located in the Head and Neck Region Treated With Platinum-based Chemotherapy
The aim of this study is to evaluate risk factors contributing to the development of ototoxicity in children who received platinum-based chemotherapy for malignancies located in the head and neck region. Eighty-four children who received platinum-based chemotherapy were included. Audiologic evaluations were performed before and after each chemotherapy session through pure tone audiometry, distortion product otoacoustic emissions, and auditory brainstem response tests. Ototoxicity was evaluated using Brock, Muenster, and Chang classifications. Factors such as cranial irradiation, cumulative doses of cisplatin, age, sex, cot...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting
Children with neuroblastoma (NB) and opsoclonus-myoclonus-ataxia syndrome (OMAS) have a favorable oncologic outcome and overall survival. In contrast, despite intensive multidrug immunomodulation, the neurologic outcome is complicated by the relapsing nature of the neurologic symptoms and long-term neurobehavioral sequelae. Being associated with low-risk NB, there exists an ambiguity in the current literature regarding the administration of chemotherapy in these children. We reviewed our archives for children with NB-OMAS over a 22-year (January 1996 to January 2018) period. Eighteen children (10 female) with a median age ...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Challenges in the Management of Childhood Intracranial Germ Cell Tumors in Middle-Income Countries: A 20-Year Retrospective Review From a Single Tertiary Center in Malaysia
Conclusions: The survival rate of childhood iGCTs in UMMC was inferior to that reported in developed countries. Late diagnosis, poor adherence to treatment, and treatment-related complications were the contributing factors. Although these results highlight a single institution experience, they most likely reflect similar treatment patterns, outcomes, and challenges in other centers in Malaysia. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Treatment of Primary Central Nervous System Germinomas With Short-course Induction Chemotherapy Followed by Low-dose Radiotherapy Without a Tumor Bed Boost: Prognostic Impact of Human Chorionic Gonadotropin
Conclusions: Some children with primary CNS germinoma may benefit from short-course induction chemotherapy followed by low-dose radiotherapy to the whole ventricle without a tumor bed boost. The validity of our findings needs to be confirmed in a randomized phase II study for children with β-hCG levels (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Cardiac Inflammatory Myofibroblastic Tumor Causing Pulmonary Artery Obstruction: A Rare Case Report
Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor of the heart. In the literature, cardiac IMT is often described as an endocardial-based cavitary mass originating from the right side of the heart in infants and adolescents. In this article, we present a 5-year-old boy with a rare cardiac IMT who had no complaints and was diagnosed with murmur during his routine examination. Transthoracic echocardiography showed a homogeneous polypoid mass originating from the pulmonary valve, extending into the main pulmonary artery during systole and causing obstruction of the pulmonary artery and right ventricular out...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

COVID-19 in Pediatric Cancer Patients: How Concerned We Should Be? Lessons Learned From a Single Center in Middle East
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Letter to the Editor Source Type: research

Acute Myeloid Leukemia Presenting as a Posterior Fossa Tumor
We report a case of acute myeloid leukemia with central nervous system-MS presenting as a posterior fossa mass mimicking a primary intracranial tumor. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Radiology Corner Source Type: research

Decision-Making in the Era of New Medical Technologies in Pediatric Hematology-Oncology: The Death of Palliative Care?
Discussion: Although health care providers individually envisioned that palliative care may be the best course of action for patients receiving emergent therapy, they remained collectively in the curative mode. The intricate relationship between science, hope, caregiver, and care receiver sustains this perspective even when (near) death is the probable outcome. When proven treatment fails, emerging therapeutic possibilities offer new hope that can delay the referral to the palliative care team. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Medical Progress Source Type: research

Comparison of Total Body Irradiation–based Versus Chemotherapy-based Conditionings for Early Complications of Allogeneic Hematopoietic Stem Cell Transplantation in Children With ALL
Conclusions: Our findings support chemotherapy-based regimens with early neutrophil engraftment, less ES and CMV reactivation compared with TBI. Although there is no impact on survival rates, increased incidence of ES and CMV reactivation should be considered in TBI-based regimens. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Original Articles Source Type: research

Mortality and Causes of Death From Sickle Cell Disease in The Netherlands, 1985-2017
In the Netherlands, between 1985 and 2007 secular changes in the health care of patients with sickle cell disease (SCD) have taken place, such as penicillin prophylaxis, vaccination programs and stroke prevention. We investigated the number and causes of death in a cohort of 298 SCD patients, established in 2007, before introduction of neonatal screening, to determine preventable deaths. All patients were diagnosed with SCD before the age of 18 (median age at diagnosis 5.1 y). Their vital status was determined up to January 2017. After a total follow-up period of 4565 patient years and a median time of follow-up of ...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Original Articles Source Type: research

Autoimmune Liver Disease in Patients With Sickle Cell Disease
Conclusions: In this cohort, there seems to be an association between SCD and AILD; SC in this population was severe. Physicians should be aware of this and evaluate patients with SCD and elevated liver enzymes for AILD. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Original Articles Source Type: research

Associations Between Transcranial Doppler Flow Velocities, Laboratory Parameters, and Clinical Features in Turkish Children With Sickle Cell Disease
Conclusions: High ferritin and low hemoglobin levels, a high mean platelet volume, a high red cell distribution width, low weight (below the third percentile), and a short height (below the third percentile) may be associated with elevated cerebral blood flow velocities and an increased stroke risk in children with SCD. Children with such features should be closely followed-up through transcranial Doppler ultrasonography examination. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Original Articles Source Type: research

Thrombocytopenia in COVID-19: Focused Summary of Current Understanding of Mechanisms and Clinical Implications
Thrombocytopenia is noted in corona virus disease-2019 (COVID-19) with a prevalence of 5% to 41%, and has been observed to be associated with inferior outcomes. The pathogenesis of thrombocytopenia in COVID-19 is unique and differs from other viral syndromes in terms of clinical presentation and causative mechanisms. Platelets act as both targets and the initial defense against severe acute respiratory syndrome-coronavirus 2 and work in concert with the underlying thrombophilic mechanisms to modulate the final disease phenotype. Understanding these mechanisms may possibly allow targeting of a key component of COVID-19 path...
Source: Journal of Pediatric Hematology Oncology - September 24, 2021 Category: Hematology Tags: Review Article Source Type: research

Unusual Clinical Presentation of Hodgkin Lymphoma in a Child: Both Spinal Cord Compression and Hydronephrosis
Conclusion: Differential diagnosis of abdominal mass in patients presenting with spinal cord compression and/or hydronephrosis should include HL. Retrograde J ureteral stenting is the treatment of choice for malignant ureteral obstruction. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Fecal Microbiota Transplantation for Treatment of Severe Clostridioides difficile Colitis in a Pediatric Patient With Non-Hodgkin Lymphoma
Conclusions: FMT may be a feasible option for treatment of refractory CDI in pediatric oncology patients. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Improving Detection of Cancer Predisposition Syndromes in Pediatric Oncology
Implementation and adherence to consensus statement criteria for referral of pediatric cancer patients for genetic evaluation are critical to identify the 5% to 10% with a genetic cancer predisposition syndrome. The authors implemented a Plan-Do-Study-Act quality improvement initiative aimed at increasing referrals of at-risk patients. Retrospective chart review was followed by educational intervention—with impact assessed over a 9-month prospective chart review. Referral rate improved>2-fold and there was an improvement in documented oncologic history to at least a third-degree relative. The integration of qualit...
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Rasburicase-induced Methemoglobinemia: A Case Report and Literature Review
Rasburicase is a recombinant urate oxidase enzyme indicated for tumor lysis syndrome, a potential life-threatening oncologic emergency that occurs most commonly during initial chemotherapy for hematological malignancies. As a result of the defects in the physiological antioxidant pathway, erythrocytes of patients with glucose-6-phosphate dehydrogenase deficiency are not protected against the oxidizing stress exerted by hydrogen peroxide generated with the administration of rasburicase. The authors report a 14-year-old patient, diagnosed with T-cell acute lymphoblastic leukemia, who developed methemoglobinemia and hemolytic...
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Assessing Safe Discharge Criteria for Pediatric Oncology Patients Admitted for Febrile Neutropenia
Recent studies suggest outpatient therapy, oral antibiotics, or earlier discharge could be appropriate in some pediatric patients admitted with febrile neutropenia; supporting data are lacking. Retrospective chart review of patients admitted from September 2005 through October 2016 identified 131 “early discharge” febrile neutropenia admissions with discharge absolute neutrophil count (ANC) (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Original Article Source Type: research

Examining the Potential Relationship Between Multidisciplinary Team Meetings and Patient Survival in Pediatric Oncology Settings: A Systematic Review
The organization of multidisciplinary team meetings (MTMs) has become standard practice in pediatric oncology and is widely felt to improve communication, knowledge, and patient care. Although the impact of MTMs on survival in adult oncology has been extensively researched, the potential benefits of survival for pediatric cancer patients are still unclear. This systematic review aimed to examine the impact of MTMs on survival in pediatric oncology settings. Relevant studies were identified by searching MEDLINE/PubMed, EMBASE, and the Cochrane Library databases up to January 2020, resulting in 325 unique records. After the ...
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Review Article Source Type: research

Is Busulfan Clearance Different in Patients With Sickle Cell Disease? Let’s Clear Up That Case With Some Controls
In busulfan-based conditioning regimen for hematopoietic stem cell transplantation in children, accurate a priori determination of the first dose is important because of its narrow therapeutic window. Sickle cell disease (SCD) influences pharmacokinetics of the commonly used drugs by affecting organs responsible for drug metabolism and elimination. This pharmacokinetics study assesses the influence of SCD on the metabolic pathway of busulfan that is mainly metabolized in the liver. In this retrospective cross-sectional case-control study, 16 patients with SCD were matched to 50 patients without SCD on known busulfan cleara...
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research