Journal of Pediatric Hematology/Oncology最新文献

{"title":"Pyrites: A Right Orbital Tumor.","authors":"Bun Sereyleak, Has Sothearak, Sam Lyvannak, Thy Bunpaov, Khauv Phara, Yin Sopheakbot, Ven Ratanak, Jason Jarzembowski, Frank Goulding Keller, Bruce Camitta","doi":"10.1097/MPH.0000000000003060","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003060","url":null,"abstract":"","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144475746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pyrites: A Chest Mass.","authors":"Sam Lyvannak, Thy Bunpaov, Bun Sereyleak, Has Sothearak, Um Khemuoy, Jason Jarzembowski, Bruce Camitta","doi":"10.1097/MPH.0000000000003062","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003062","url":null,"abstract":"","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144475745","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"DRT (Delayed Rewarming Thrombocytopenia) Not DIC, is the Cause of Rewarming Deaths in Neonatal Cold Injury.","authors":"Ian J Cohen","doi":"10.1097/MPH.0000000000003065","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003065","url":null,"abstract":"<p><p>DIC was suspected of being the cause of fatal rewarming deaths in neonatal cold injury (NCI) in the absence of any other known explanation for this complication. It was associated with thrombocytopenia, bleeding, and abnormal clotting studies. Subsequently, this suspicion was questioned because of the reversibility of thrombocytopenia and more recently because of the delineation of a new entity DRT (delayed rewarming thrombocytopenia) that better explained the clinical and laboratory features of this condition. The sudden bleeding that occurs after 24 hours of hypothermia is explained by the reappearance, on rewarming, of the second stage of (irreversible) platelet aggregation that does not occur below 32°C, and the presence of high levels of ADP that leak from erythrocytes. It is recommended that DRT should be treated by rapid rewarming, blocking of the second phase of platelet aggregation or platelet transfusions rather than replacement therapy with several clotting factors that would be appropriate in DIC.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adolescent and Young Adult Oncology in the United States in 2025: Finding Its Place in the Oncology World Part 2 of 2.","authors":"Peter H Shaw, Sharon M Castellino, Ryan Guerrettaz, Nicholas Yeager, Bradley Zebrack, Rachel Brandon, Archie Bleyer","doi":"10.1097/MPH.0000000000003066","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003066","url":null,"abstract":"<p><p>In 2015, this core of authors wrote a \"state of the union\" overview of AYA oncology care at the time titled \"Adolescent and Young Adult (AYA) Oncology in the United States: A Specialty in Its Late Adolescence.\" Since then, the landscape of cancer care in this unique population has changed, with encouraging improvement in some areas and persistent challenges in others. Ten years later, we have decided to update our review to demonstrate how far we have come in caring for 15 to 39-year-olds with cancer in the United States and how much further we need to go to truly improve both their short and long-term outcomes. Back in 2015, we described the field as in its late adolescence, still trying to define itself. With this 2-part review, we hope to demonstrate that as a subspecialty, it has grown up but is still trying to firmly establish its place in the larger world of oncology, much like a young adult that has moved away from home and is establishing its own identity in a changing world. In the first part of our update on the state of adolescent and young adult (AYA) oncology in the United States in 2025, we reviewed the epidemiology of AYA cancers (those in 15 to 39-year-olds) as well as the advancements in the management of acute lymphoblastic leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma, and sarcomas in this population. In this second part, we review the topics of clinical trial enrollment, models of care, the psychosocial impact of cancer in this population and survivorship.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Critical Complication in Childhood Leukemia: Neutropenic Enterocolitis, Risk Factors, and Outcomes.","authors":"Ayca Koca Yozgat, Rabia D Kilçik, Selin Çetin, Buket Altintaş, Dilara Aydoğdu, Merve İlsol, Fatma B Kurtipek, Çiğdem Bulut, Melek Işik, Dilek Kaçar, Özlem Arman Bilir, Neşe Yarali","doi":"10.1097/MPH.0000000000003064","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003064","url":null,"abstract":"<p><strong>Objective: </strong>Neutropenic enterocolitis is characterized by inflammation of the cecum and distal ileum. The clinical triad consists of fever, abdominal pain, neutropenia, and imaging findings of colonic inflammation. Herein, we report the clinical characteristics, imaging findings and risk factors of typhlitis in pediatric patients with leukemia.</p><p><strong>Methods: </strong>In this retrospective study, 72 pediatric patients who were followed up due to acute lymphoblastic or myeloblastic leukemia and diagnosed with typhlitis were evaluated between August 2019 and March 2023. Typhlitis was diagnosed based on the presence of neutropenia (absolute neutrophil count [ANC] <500/mm³ or an ANC expected to decrease to <500/mm³ within the next 48 h), at least one clinical symptom or sign, and confirmatory imaging showing cecum wall thickness >3 mm on ultrasonography. Patients' data regarding age, sex, chemotherapy regimens, clinical symptoms, port/catheter and peripheral blood cultures, imaging findings, comorbidities, length of hospital stay, management of typhlitis, and mortality rates were evaluated.</p><p><strong>Results: </strong>Neutropenic enterocolitis was diagnosed in 72 children based on clinical and imaging features with an incidence of 36% (5 patients had recurrent episodes). Common manifestations included abdominal pain (81.8%), fever (48.1%), and diarrhea (42.9%). The most isolated organism in patients' port catheter and peripheral blood cultures was Klebsiella pneumonia. The most frequently affected bowel segments as seen on ultrasonograms were the cecum and terminal ileum (37.7%). The mean wall thickness was 4.6±1.7 mm. The median duration of intravenous broad-spectrum antibiotic treatment in our patients was 13 days (range: 8 to 24). The mean time to resume chemotherapy in our patients was 13.6±15.5 days. The median hospital stay of patients due to neutropenic enterocolitis was prolonged by 12 days. Surgical intervention was performed in three patients due to intestinal perforation. Four patients (5%) died due to neutropenic enterocolitis and sepsis. Patients who died from typhlitis and sepsis had a longer duration of typhlitis, although this difference was not statistically significant in terms of mortality rates.</p><p><strong>Conclusion: </strong>Neutropenic enterocolitis is a complex clinical challenge that requires early diagnosis and a multidisciplinary approach. It should be suspected in any patient who developed neutropenia because of chemotherapy and presents with gastrointestinal symptoms such as nausea, vomiting, diarrhea, and severe abdominal pain.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Exocrine Pancreatic Insufficiency Following Asparaginase-induced Pancreatitis in Pediatric Acute Lymphoblastic Leukemia Patients: A Case Series.","authors":"Rachel Hill, Lindsey Thomas, David Farbo, Kenneth Heym","doi":"10.1097/MPH.0000000000003058","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003058","url":null,"abstract":"<p><p>Asparaginase is vital for the treatment of pediatric acute lymphoblastic leukemia (ALL), but it can cause numerous adverse effects, including asparaginase-associated pancreatitis (AAP). Exocrine pancreatic insufficiency (EPI) has been reported following pancreatitis but has not yet been reported in the setting of pediatric leukemia. If undiagnosed, EPI can lead to nutritional deterioration. This series describes 3 cases of severe, necrotizing AAP followed by EPI of variable duration. The purpose of this case series is to document these findings and increase awareness about the association between these conditions to facilitate timely identification and intervention, when warranted.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248391","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ovarian Mitotically Active Cellular Fibroma of Childhood: Review of Literature and Report of a Case.","authors":"Aileen Azari-Yam, Sahar Montazeri, Bahar Ashjaei, Soheila Sarmadi","doi":"10.1097/MPH.0000000000003059","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003059","url":null,"abstract":"<p><p>Ovarian fibromas, especially those of the mitotically active cellular subtype, are very rare in children; however, children with nevoid basal cell carcinoma syndrome (NBCCS) are prone to these neoplasms. We present a 12-year-old girl with an ovarian mitotically active cellular fibroma (MACF) with no personal or family history of NBCCS who underwent right salpingo-oophorectomy. We searched the English literature on pediatric ovarian MACF. To date, only 3 cases of MACF in children have been reported. We present a pediatric case that represents the youngest reported case of MACF thus far. Special attention is required to differentiate this tumor from its malignant mimics to avoid overtreatment, such as radical surgeries. Long-term follow-up and thorough investigation of the manifestations of hereditary cancer syndromes should be considered.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248392","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Capturing Fertility: A Qualitative Exploration of Retention of a Fertility Consult in Sickle Cell Disease.","authors":"Mahvish Q Rahim, Derrick L Goubeaux, Christina Ebenroth, Aikaterini Voulgaridou, Seethal A Jacob, Amanda J Saraf","doi":"10.1097/MPH.0000000000003056","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003056","url":null,"abstract":"<p><p>This qualitative study assessed the perceptions of caregivers of adolescents with sickle cell disease regarding fertility preservation consultation before stem cell transplant, through semistructured interviews. We interviewed 7 caregiver-adolescent dyads and 1 caregiver whose child didn't meet inclusion criteria due to age. Thematic analysis revealed 3 major inter-related themes: burden of sickle cell disease, decisional regret about reproductive choices, and hope that infertility would not impact them. Our study found that comprehension about the potential for infertility varied significantly, with a strong underlying hope that infertility will not impact them. Many of our caregivers and adolescents indicated decisional regret, suggesting they would now make a different choice about fertility preservation before transplant. Conversations and communication surrounding infertility in the setting of stem cell transplant is vital for our patients to understand the long-term impacts of curative therapy, to best ensure that their long-term quality of life goals will be met. Though the recent movement towards reduced intensity conditioning regimens in SCT may prove less gonadotoxic, fertility outcome data are not yet known. This study underscores the importance of effective communication during dedicated fertility consultations to help families make informed decisions for their children.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248327","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Concurrent Case of Glucose-6-Phosphate Deficiency and Dehydrated Hereditary Stomatocytosis in a 4-Month-old Boy.","authors":"Thao Minh Nguyen, Sara Sadiq, Joshua M Peterson, Leonard K Wang, Gulrukh Botiralieva, Yaroslav Chernov, Akila Muthukumar, Kirill A Lyapichev","doi":"10.1097/MPH.0000000000003057","DOIUrl":"https://doi.org/10.1097/MPH.0000000000003057","url":null,"abstract":"<p><p>Glucose-6-phosphate (G6PD) deficiency is the most prevalent enzyme deficiency and is estimated to affect 400 million people. The patients are usually asymptomatic and diagnosed following hemolytic episodes triggered by oxidative stress. Another type of hemolytic anemia known as dehydrated hereditary stomatocytosis (DHSt) is estimated to affect less than 1 per 1,000,000 people. DHSt is caused by increased cation efflux and dehydration in red blood cells, which leads to decreased flexibility making them more vulnerable to lysis. Compared with G6PD, DHSt has a mild presentation, where most patients (84%) with isolated DHSt exhibit chronic hemolysis. Both diseases, G6PD deficiency and DHSt, are inherited hemolytic anemias and to the best of our knowledge have never been reported to coexist in the same patient. Herein, we present the first case of concurrent G6PD deficiency and DHS in a 4-month-old male. We discuss the clinical presentation and hematopathology findings from this patient as well as provide a comparison literature review. We believe this presentation will add to the current body of knowledge for these conditions and help to guide future investigation and management.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2025-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144248389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sustained Clinical Remission of BRAF V600E-Mutated Langerhans Cell Histiocytosis With Multiorgan Involvement in an Infant Treated With Dabrafenib.","authors":"Franziska Cuntz, Jean Donadieu, Susanne Holzhauer","doi":"10.1097/MPH.0000000000003014","DOIUrl":"10.1097/MPH.0000000000003014","url":null,"abstract":"<p><strong>Background: </strong>Above 50% of LCH cases show BRAF-mutations, which can be targeted by dabrafenib in refractory disease.</p><p><strong>Observations: </strong>Here, we report on a patient with neonatal multisystem, BRAF-mutated LCH refractory to conventional treatment with vinblastine and prednisolone. Duodenal involvement rendered oral nutrition impossible, and the patient was severely ill with pancytopenia, hepatic dysfunction, cholestasis, and septic episodes. After initiation of targeted therapy with dabrafenib, the patient achieved sustained clinical remission.</p><p><strong>Conclusions: </strong>Multisystem LCH is a rare and potentially life-threatening disease that can mimic various neonatal conditions. A high index of suspicion is necessary for diagnosis. Timely initiation of targeted therapy may prevent irreversible organ damage.</p>","PeriodicalId":16693,"journal":{"name":"Journal of Pediatric Hematology/Oncology","volume":" ","pages":"210-213"},"PeriodicalIF":0.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}