Pediatric Benign Neutropenia: Assessing Practice Preferences in Canada
Pediatric benign neutropenia is a self-limited condition with a benign clinical course. An approach to this condition is not well-defined in the literature. Our objective was to use a case-based survey to elucidate trends in the diagnosis and management of benign neutropenia among pediatric hematology/oncology practitioners in Canada. We received 46 completed surveys (response rate 66%). At initial presentation with fever and neutropenia, 67% of respondents recommended partial septic workup but 11% recommended no investigations. Nearly 70% recommended admission for empiric intravenous antibiotics, while 24% would discharge...
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Considerations in Methods and Timing for Delivery of Genetic Counseling Information to Pediatric Oncology Patients and Families
In conclusion, patients and their families may benefit from prompt and early recognition of the risk of cancer predisposition syndromes, preferably within the first 2 months following diagnosis. Oncology professionals are an important source of information, and can introduce the availability of genetic counseling services and motivate families to undergo genetic testing, though alternative communication methods such as brochures may also be useful. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Is Detection of Relapse by Surveillance Imaging Associated With Longer Survival in Patients With Rhabdomyosarcoma?
Conclusion: These results on whether surveillance imaging provides survival benefit in patients with relapsed RMS are inconclusive. Larger studies are needed to justify current surveillance recommendations. Chemotherapy, radiotherapy and surgery to treat recurrence prolong OS. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Multifarious Functions of Butyrylcholinesterase in Neuroblastoma: Impact of BCHE Deletion on the Neuroblastoma Growth In Vitro and In Vivo
The physiological functions of butyrylcholinesterase (BChE) and its role in malignancy remain unexplained. Our studies in children newly diagnosed with neuroblastoma indicated that BChE expressions is proportional to MYCN amplification suggesting that pathogenesis of high-risk disease may be related to the persistent expression of abnormally high levels of tumor-associated BChE. BChE-deficient neuroblastoma cells (KO [knockout]) were produced from MYCN-amplified BE(2)-C cells (WT [wild-type]) by the CRISPR-Cas9 targeted disruption of the BCHE locus. KO cells have no detectable BChE activity. The compensatory acetylcholines...
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

Longer Time Intervals From Symptom Onset to Diagnosis Affect the Overall Survival in Children With Acute Lymphoblastic Leukemia
Conclusions: These findings suggested that a longer time to diagnosis negatively affected the clinical outcome of childhood ALL. Reducing the time to diagnosis could help to improve survival in these patients. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Original Articles Source Type: research

New Approaches Promise to Improve Local Ewing Sarcoma Results
The study by Whelan and colleagues showed that addition of busulfan and melphalan conditioning and autologous stem cell rescue to conventional EURO-E.W.I.N.G STUDY chemotherapy in local nonmetastatic Ewing sarcoma improves prognosis. However, almost 30% of these study patients will have relapsed before this stage of therapy is reached, and 78% of his patients were at high risk because of inadequate response to the initial chemotherapy given. Further improvement could be achieved by the integration of other novel advances with this approach. Ash and colleagues have shown that the separation of such cases into high- and low-...
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Medical Progress Source Type: research

Drug Induced Diabetes Mellitus in Pediatric Acute Lymphoblastic Leukemia: Approach to Diagnosis and Management
Corticosteroids and l-asparaginase used in the treatment of pediatric acute lymphoblastic leukemia (ALL) can cause drug-induced diabetes mellitus (DIDM). DIDM can lead to dyselectrolytemia, a higher risk of infections including cellulitis, bacteremia, fungemia, and a higher incidence of febrile neutropenia and may have an impact on the outcome of ALL. Literature on the management of DIDM among children with ALL is sparse and the diagnostic criteria for pediatric diabetes should be carefully applied considering the acute and transient nature of DIDM during ALL therapy. Insulin remains the standard of care for DIDM managemen...
Source: Journal of Pediatric Hematology Oncology - August 1, 2022 Category: Hematology Tags: Review Article Source Type: research

Leiomyomatosis in an Infant With a SUFU Splice Site Variant: Case Report
We describe a case of a 5-month-old female who presented with multiple intra-abdominal leiomyomata and was found to have a likely pathogenic splice site variant in the SUFU gene. This is the first reported case of leiomyomatosis secondary to a pathogenic SUFU variant in an infant and may represent an early, atypical presentation of Gorlin syndrome. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

An Unusual Presentation of Sacrococcygeal Germ Cell Tumor With Autoimmune Hemolytic Anemia as a Paraneoplastic Syndrome
We report an unusual presentation of AIHA with malignant sacrococcygeal germ cell tumor in a 3-year-old child, which rapidly disappeared after the excision of a sacrococcygeal teratoma. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Hemophagocytic Lymphohistiocytosis Associated With Hemolytic Uremic Syndrome in a Child: A Case Report and Systematic Literature Review
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder resulting from excessive activation and nonmalignant proliferation of T-lymphocytes and macrophages. Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Reports pertaining to the association between HLH and HUS are rarely published; however, we report on a 4-year-old boy who was diagnosed with both conditions and treated successfully with high-dose steroid and intravenous immunoglobulin. Differentiating HUS from HLH can be challenging because of their clinical simil...
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Do Not Forget About the Ticks: An Unusual Cause of Fever, GI Distress, and Cytopenias in a Child With ALL
We report the case of a 5-year-old male with B-cell acute lymphoblastic leukemia in remission, receiving maintenance chemotherapy, who presented with fever, emesis, diarrhea, headache, and lethargy. He developed rapidly progressive cytopenias and was found to have acute human granulocytic anaplasmosis as well as evidence of past infection with Babesia microti. The case highlights the need to maintain a broad differential for infection in children undergoing chemotherapy or other immunosuppressive therapies with possible or known tick exposure. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Bortezomib-based Anthracycline-free Induction for Pediatric Relapsed ALL as a Bridge to Immunotherapy
Conclusion: Anthracyclines can be safely omitted from bortezomib-based therapies in patients with R/R ALL, when planning to proceed to immunotherapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Original Article Source Type: research

Affirming Hormone Treatment for a Transgender Adolescent After a Venous Thromboembolic Event
Conclusions: The possibility of testosterone therapy as a risk factor for VTE may suggest the need to include this information during informed consent discussions. Long-term anticoagulation may be considered for those restarting testosterone therapy. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Successful Use of Thrombolysis in an Extremely Low Birth Weight, Premature Infant With Aortic Thrombosis
Conclusion: Alteplase may be considered in premature, extremely low–birth weight infants with careful assessment of risk and benefits, along with frequent surveillance and supportive care. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Identifying and Treating Severe Bone Marrow Necrosis and Fat Embolism Syndrome in Pediatric Patients With Sickle Cell Disease: A Case Report
We report a pediatric patient who was diagnosed early, received urgent red cell exchange transfusion and plasma exchange, and ultimately survived this devastating complication. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - June 30, 2022 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research