Journal of Pediatric Hematology Oncology 
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This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.Vaso-occlusive Pain Crisis Mimicking Mandibular Abscess in an Adolescent with Sickle Cell Disease
We present an unusual case of a 14-year-old girl with SCD whose initial presentation of facial swelling, headaches, jaw pain, and paresthesia mimicked an odontogenic abscess. She was diagnosed with vaso-occlusive crisis in the mandibular bone and successfully managed noninvasively. This is among the youngest cases of paresthesia in the lower lip in SCD, which provided a clue that postponing invasive aspiration or biopsy was possible under empiric antibiotics and close observation. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Two Cases of Acute Direct Oral Anticoagulant Overdose Without Adverse Effect
We report 2 pediatric patients who had acute overdoses of the direct oral anticoagulants medications. Both patients were managed conservatively; neither required reversal agents or blood products nor had any major or minor bleeding events. With therapeutic usage of direct oral anticoagulants, routine coagulation studies typically are considered insufficient measures of anticoagulation and the preferred chromogenic anti-Factor Xa assay is recommended but not widely available. Using a routine hybrid heparin anti-Factor Xa assay, 1 patient demonstrated a strong linear correlation up to a serum rivaroxaban concentration of 940...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
A 17-Month-old Boy With Pancytopenia Caused by a Rare Genetic Defect of Vitamin B12 Malabsorption
We present the case of a 17-month-old boy with failure to thrive, pancytopenia, and fevers. His megaloblastic anemia was overlooked leading to unnecessary invasive testing. Findings on bone marrow biopsy prompted investigation for genetic disorders of B12 metabolism. Exome sequencing uncovered 1 known pathogenic variant and 1 novel likely pathogenic variant in CUBN, confirming the diagnosis of Imerslund-Gräsbeck syndrome. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
A Case of a Pediatric Patient With Protein S Heerlen Polymorphism and Deep Venous Thrombosis
We report a case of an adolescent female with May Thurner syndrome and heterozygous PS Heerlen mutation resulting in a mild PS deficiency and venous thromboembolism. With this nonmodifiable risk factor, the patient received prolonged anticoagulation with strong consideration for lifelong prophylaxis. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Genetic Study of Fanconi Anemia in Infancy Revealed FANCI Mutations and Defective ALDH2 Variant: A Case Report
Fanconi anemia (FA) is a rare genetic disorder that manifests as congenital abnormalities and bone marrow failure (BMF). Most patients with FA present with BMF within the first decade of life; however, neonate and early infancy BMF is rare. Recent studies have shown that a defective aldehyde dehydrogenase 2 (ALDH2) variant accelerates BMF development in patients with FA. Herein, we described an infant case of FA with compound heterozygous FANCI mutation and the defective ALDH2 variant. Our case developed BMF early probably because of ALDH2 deficiency, while the mild malformation might be because of the locus of FANCI mutat...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Acquired Thrombotic Thrombocytopenic Purpura in a 5-Year-old Child With Wiskott-Aldrich Syndrome
Thrombocytopenia is often seen as a laboratory finding during childhood. A supposed idiopathic thrombocytopenic purpura patient who was later diagnosed as Wiskott-Aldrich syndrome (WAS) and developed acquired thrombotic thrombocytopenic purpura (aTTP). Although autoimmune manifestations in WAS described, aTTP was reported just once. Five-year-old-boy was initially brought with cough, bloody stool (diarrhea), oral mucosal bleeding at 12th months of age. Following diagnosed with idiopathic thrombocytopenic purpura and receiving intravenous immunoglobulin, platelet count raised from 20,000 to 50,000/µL. One year after WAS di...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Amyloidosis in a Patient With Congenital Neutropenia Because of G6PC3 Deficiency
Glucose-6-phosphatase catalytic subunit 3 (G6PC3) deficiency is a recently identified form of congenital neutropenia associated with developmental anomalies. The severity of neutropenia and the clinical spectrum are highly variable. Aside from infectious complications and extrahematologic features, inflammatory bowel disease and autoinflammatory complications are less frequently observed manifestations. However, amyloidosis has never been reported in G6PC3 deficiency. Here, we present a 12-year-old patient with incidentally discovered neutropenia because of the p.E65A (c.194A>C) variant of the G6PC3 gene. He had recurrent ...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Multifocal Osteonecrosis in a 3-Year-old Child With Sickle Beta Plus Thalassemia
Sickle beta+thalassemia is considered to be a mild form of sickle cell disease. However, some patients with mild disease can present with osteonecrosis. Here, we present a rare 3-year-old male who presented with acute pain, a baseline hemoglobin of 13 g/dL, who acutely developed multifocal osteonecrosis, and improved with partial exchange transfusion and hydroxyurea therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Successful Management of Massive Congenital Hepatic Hemangioma and Systemic Hypertension With Sirolimus
Congenital hepatic hemangioma (CHH) is a common benign vascular tumor of the liver, seen in infancy. The clinical manifestations vary from incidental findings to life-threatening complications. The authors present here a case of an infant with massive CHH who developed systemic hypertension because of compression of the right renal artery by the CHH and did not respond to other lines of treatment. After sirolimus therapy, the CHH size decreased and antihypertensive drugs were no longer necessary. In a critical situation, if the embolization and/or steroids do not seem to control the situation, then adding sirolimus may be ...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
A Novel Homozygous HAX1 Mutation in a Child With Cyclic Neutropenia: A Case Report and Review
Conclusion:
The patient extends the clinical variability associated with HAX1 variants and highlights the importance of genetic investigations in patients with suspected cyclic neutropenia. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research
Large Anterior Mediastinal Mass and Cardiac Infiltration at Diagnosis in a Child With T-cell Acute Lymphoblastic Leukemia
We report a 4-year-old female with new-onset T-cell acute lymphoblastic leukemia who was found to have a large interventricular septal mass upon initial presentation. This patient required a unique management approach with serial echocardiograms, continuous telemetry, and hemodynamic monitoring with close surveillance for ventricular ectopy. Given a good response to traditional leukemia therapy, the cardiac mass was presumed to have been a focal infiltrate of leukemic cells. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Radiology Corner Source Type: research
An Assessment on the Effectiveness of the Immature Granulocyte Percentage in Predicting Internal Organ Involvement Among Children With Henoch-Schönlein Purpura
This study included 75 patients below 18 years old who were diagnosed with HSP. The mean age was 7.48±2.77 years. The male/female ratio was 1.14. The findings showed that 35 (46.7%) of the patients had an internal organ involvement. The mean IG percentage was 0.88±0.68 among the patient group with HSP internal organ involvement, while it was 0.31±0.15 in the group without internal organ involvement, and a significant difference was determined between the 2 groups (P=0.000). The findings showed that the patients with renal involvement had the highest mean IG percentage (IG; 1.00±0.21). When the cutoff value for the IG p...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research
Outcomes of Lymphoma Among American Adolescent and Young Adult Patients Varied by Health Insurance—A SEER-based Study
This study aimed to clarify associations between health insurance status and overall survival (OS) estimates in this population.
Materials and Methods:
We examined 18 Surveillance, Epidemiology, and End Results registries in the United States and analyzed American AYA patients with lymphoma diagnosed during January 2007 and December 2016. Health insurance status was categorized, and Kaplan-Meier and multifactor Cox regressions were adopted using hazard ratio and 95% confidence interval. Probable baseline confounding was modulated by multiple propensity score.
Results:
A total of 21,149 patients were considere...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research
Assessment of Minimal Residual Disease in Childhood Acute Lymphoblastic Leukemia: A Multicenter Study From Turkey
Assestment of minimal residual disease (MRD) in childhood acute lymphoblastic leukemia (ALL) is of utmost importance both for risk classification and tailoring of the therapy. The data of pediatric ALL patients that received treatment with Berlin-Frankfurt-Münster (BFM) protocols were retrospectively collected from 5 university hospitals in Turkey. Of the 1388 patients enrolled in the study 390 were treated according to MRD-based protocols. MRD assestment was with real time quantitative polymerase chain reaction (qPCR) in 283 patients and with multiparametric flow cytometry (MFC)-MRD in 107 patients. MRD monitoring had up...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research
Clinical Significance of MicroRNA-29a and MicroRNA-100 Gene Expression in Pediatric Acute Myeloid Leukemia
Aim:
The aim of this study was to evaluate the diagnostic and prognostic performance of miRNA-29a and miRNA-100 in pediatric acute myeloid leukemia (AML).
Patients and Methods:
In all, 73 children with diagnosed pediatric AML (based on standard morphologic, cytochemical, cytogenetic, immunologic, and molecular workup, and the French-American British classification) admitted to Children’s Cancer Hospital Egypt (CCHE-57357), and 9 healthy age-matched and sex-matched controls were recruited for a case-control study. Gene expression levels of miRNA-29a and miRNA-100 were assessed using real-time quantitative RT-PC...
Source: Journal of Pediatric Hematology Oncology - February 24, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research
