Journal of Pediatric Hematology Oncology 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.

Soft Tissue and Visceral Organ Sarcomas With BCOR Alterations
Sarcomas with BCOR alteration are a heterogenous group characterized by changes including internal tandem duplications (ITDs) and recurring fusions with CCNB3, ZC3H7B, and other rare partners. With widespread genomic testing, these alterations are now associated with histologies such as Ewing-like sarcoma (BCOR::CCNB3), high-grade endometrial stromal sarcoma (ZC3H7B::BCOR), and clear cell sarcoma of kidney (BCOR-ITD). BCOR altered sarcomas of soft tissues and organs were identified through PubMed using keywords “Sarcoma (AND) BCOR” from 2005 through October 2021. Summary statistics and outcome data were calculated usin...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Review Articles Source Type: research

A Case of Gorlin-Goltz Syndrome Without the Characteristic Physical Features That Was Diagnosed After the Development of a Fifth Cancer
We present a case of Gorlin-Goltz syndrome (GGS) in a patient who developed medulloblastoma, osteosarcoma, myelodysplastic syndrome, basal cell carcinoma, and odontogenic keratocyst by the age of 19 years. He had no known family history and no characteristic physical features of GGS. A frameshift mutation in the PTCH1 gene was found in the oral mucosa as a low-frequency mosaicism, basal cell carcinoma, and normal skin by whole exome sequencing of cancer susceptibility genes. Setting a therapeutic strategy with regard to second cancer development is important for pediatric cancer patients who have a background of cancer pre...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Familial Hemophagocytic Lymphohistiocytosis With Heterozygous STX11 and Homozygous UNC13D Mutations Diagnosed in the Neonatal Period
Patients with primary hemophagocytic lymphohistiocytosis may present with different mutations and phenotypic findings. It is usually presented as case reports because of its rare occurrence. Here, we discuss a case diagnosed with familial hemophagocytic lymphohistiocytosis 3, that presented in the neonatal period and was detected to have homozygous UNC13D and heterozygous STX11 mutations. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Guideline for Children With Cancer Receiving General Anesthesia for Procedures and Imaging
Children with cancer and those undergoing hematopoietic stem cell transplantation frequently require anesthesia for imaging as well as diagnostic and therapeutic procedures from diagnosis through follow-up. Due to their underlying disease and side effects of chemotherapy and radiation, they are at risk for complications during this time, yet no published guideline exists for preanesthesia preparation. A comprehensive literature review served as the basis for discussions among our multidisciplinary panel of oncologists, anesthesiologists, nurse practitioners, clinical pharmacists, pediatric psychologists, surgeons and child...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Navigating Hemostasis of Bleeding Among Children With β-Thalassemia
Bleeding phenotype is reported in β-thalassemia patients. However, the underlying etiology remains elusive. We aimed to assess coagulation profile and the platelet aggregation in β-thalassemia children with bleeding diathesis. Fifty β-thalassemia children with a positive bleeding history were recruited. Bleeding phenotype was explored through full history taking and thorough clinical examination. Complete blood count, prothrombin time, international normalized ratio, and platelets aggregometry were performed for children with negative workup. Mucosal bleeding was manifest among most of our patients (96%). Two-third of p...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Management of Peripheral T-Cell Lymphoma in Children and Adolescents Including STAT 3 Mutation Hyper-IgE Syndrome: One Size Does Not Fit All
Peripheral T-cell lymphoma (PTCL) is a rare form of lymphoma in children with limited published data on treatment and lack of a uniformly accepted treatment algorithm. We retrospectively analyzed the data in children up to 18 years of age diagnosed to have PTCL from January 2016 to June 2020. The study included six children with a median age of 10 years, the youngest being a 7-month-old girl. According to the WHO-PTCL classification, three had PTCL-not otherwise specified (NOS), 2 had hepatosplenic TCL, and 1 had subcutaneous panniculitis-like TCL. All children had presented with advanced disease, 4 in St. Jude stage IV, 2...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Prognostic Implication of DNA Methylation Signature in Atypical Choroid Plexus Papilloma With Intracranial Dissemination
An underestimation of pathologic diagnosis could be expected if disseminated choroid plexus tumors (CPTs) are diagnosed as lower grade tumors. Thus, molecular diagnosis using genome-wide DNA methylation profiling may be useful for clarifying the malignant potential of the tumor entity. Herein, we report a 2.7-year-old girl of pathologically atypical choroid plexus papilloma with intracranial dissemination. She was treated without radiotherapy and has been well, without recurrence for 32 months following the diagnosis. Subsequently, after a year from the diagnosis, T-stochastic neighbor embedding analysis was performed on m...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

The Mutation of CD27 Deficiency Presented With Familial Hodgkin Lymphoma and a Review of the Literature
This study aimed to report 4 siblings with CD27 deficiency presented with Hodgkin lymphoma. The father of the family, his 2 wives, and 17 children born from these wives were included into the study. CD27 mutation of all the family members with, and without Hodgkin lymphoma were studied. The variants detected by the exome sequencing analysis were verified by Sanger sequencing and analyzed using SeqScape Software 3. It was determined that both the father of the family and his 2 wives carried the same variant heterozygously. Of the children born to the first mother, 2 children were normal, 3 were heterozygous and 5 were homoz...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Age of Transition Readiness of Adolescents and Young Adults With Chronic Diseases in Oman: Need an Urgent Revisit
The objective of the study is to indirectly determine the appropriateness of present cut-off age of transfer by studying readiness for transition among Omani patients suffering from chronic hematological conditions. Methods: One hundred fifty adolescents and young adults with chronic hematological conditions were recruited from pediatric and adults clinics at Sultan Qaboos University Hospital. Participants were interviewed by a trained research assistant using the Arabic version of UNC TRxANSITION Scale to assess self-management skills and health related knowledge for transition. The score range is 0 to 10; the tran...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Online Articles: Original Articles Source Type: research

Suspected Drug-induced Liver Injury Due to 6-Mercaptopurine With a Superimposed SARS-CoV-2 Infection in a Patient With B-ALL: Erratum
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Errata Source Type: research

Establishing Sickle Cell Disease Stroke Prevention Teams in Africa is Feasible: Program Evaluation Using the RE-AIM Framework: Erratum
No abstract available (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Errata Source Type: research

Puzzling (IRIDA-Like and Hemolytic) Anemia in a Child With Idiopathic Pulmonary Hemosiderosis
Before the diagnosis of idiopathic pulmonary hemosiderosis (IPH), unexplained or puzzling anemia may precede and delay in the diagnosis of pediatric IPH is common. A 5.8 years old female child initiated with iron-refractory iron deficiency anemia-like iron deficiency and hemolytic anemia and at 6.8 years of age IPH was materialized, when the patient showed the triad signs of IPH with hemosiderin-laden alveolar macrophages in gastric aspirate. Although time to the diagnosis was previously reported to be ranged from 16 to 30 months, in our case it took 12 months from the initial anemia to IPH diagnosis. (Source: Journal of P...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Hodgkin Lymphoma in Children Under 5 Years: Do They Behave Differently?
We report the outcome of children under 5 years of age enrolled in the InPOG-HL-15-01, the first multicentric collaborative study for newly diagnosed children and adolescents with HL from India. Thirty-five (9%) of the study population was younger than 5 years with a striking male preponderance of 34:1. They were less likely to have bulky disease, mediastinal or splenic involvement. The outcomes appear to be at least as favorable as in the older patient group. Efforts need to be made to evolve treatment strategies that spare this very young cohort from potential late effects. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Hematologic Complications Associated With Intra-arterial Chemotherapy for Retinoblastoma Treatment: A Single Institution Experience
The objective of this retrospective study was to analyze baseline characteristics, efficacy, and hematologic complications associated with IAC treatment in children with RB at a single international referral institution. Ninety-five sessions of IAC were performed in 28 patients. Mean age at RB diagnosis was 12.5 months (SD, 9.2 mo). Fourteen patients had bilateral RB. IAC agents included melphalan, carboplatin, and topotecan. The most common regimens were triple-agent IAC and single-agent melphalan (66.3% and 15.8%, respectively). Median number of IAC sessions was 3 (mean: 3.39, range: 1 to 9). Eye salvage rate was 83.7%...
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Gemtuzumab Ozogamicin Followed by Unrelated Cord Blood Transplantation With Reduced-intensity Conditioning for a Child With Refractory Acute Promyelocytic Leukemia
We report here a case of an 8-month-old girl with APL who failed standard ATRA-combined chemotherapy. Although molecular remission was achieved after introducing ATRA/ATO combination therapy, molecular relapse occurred during the ATO consolidation courses. Subsequent molecular remission was rapidly achieved after administering 2 doses of gemtuzumab ozogamicin. She was successfully treated with unrelated cord blood transplantation using reduced-intensity conditioning. Gemtuzumab ozogamicin might be a preferable choice for patients with APL refractory to standard therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - April 27, 2022 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research