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Ewing sarcoma of the hands and feet: Outcome and prognostic factors of a rare subsite in a low-middle income country 手和脚的尤文氏肉瘤:在中低收入国家的一种罕见亚位点的结局和预后因素
Pediatric Hematology Oncology Journal Pub Date : 2025-03-01 DOI: 10.1016/j.phoj.2025.02.004
Shuvadeep Ganguly , Archana Sasi , Chitrakshi Nagpal , Bivas Biswas , Sandeep Agarwala , Deepam Pushpam , Ahitagni Biswas , Venkatesan Sampath Kumar , Love Kapoor , Shah Alam Khan , Vishesh Jain , Sameer Bakhshi
{"title":"Ewing sarcoma of the hands and feet: Outcome and prognostic factors of a rare subsite in a low-middle income country","authors":"Shuvadeep Ganguly ,&nbsp;Archana Sasi ,&nbsp;Chitrakshi Nagpal ,&nbsp;Bivas Biswas ,&nbsp;Sandeep Agarwala ,&nbsp;Deepam Pushpam ,&nbsp;Ahitagni Biswas ,&nbsp;Venkatesan Sampath Kumar ,&nbsp;Love Kapoor ,&nbsp;Shah Alam Khan ,&nbsp;Vishesh Jain ,&nbsp;Sameer Bakhshi","doi":"10.1016/j.phoj.2025.02.004","DOIUrl":"10.1016/j.phoj.2025.02.004","url":null,"abstract":"<div><h3>Background</h3><div>The small bones of the hand and feet represent a rare site of Ewing sarcoma (ES) origin. This study presents a real-world dataset describing the clinical presentation, survival outcomes, and their determinants in this subsite.</div></div><div><h3>Methods</h3><div>This is a single-institutional retrospective study of patients with ES originating from the hands/feet (ES-HF), treated between 2003 and 2018. Clinical/demographic details and survival outcomes were retrieved from medical records. Descriptive statistics were used to summarize baseline characteristics. Prognostic factors for event-free survival (EFS) and overall survival (OS) were identified by Cox regression. The clinical features and outcomes were compared between ES-HF and ES-others (ES from other sites) in the cohort.</div></div><div><h3>Results</h3><div>Of 859 ES cases, 28 (3.2 %) patients had ES-HF including four ES-hands (0.5 %) and 24 ES-feet (2.8 %). The calcaneum was the most common site [11 of 28 cases; 39.3 %]. More than half of the patients (53.6 %) had metastatic disease at presentation. In comparison with ES-others, ES-HF had longer median symptom duration [12 versus 4 months; p &lt; 0.001] and smaller tumor diameter [5.5 versus 9 cm; p &lt; 0.001]. The median EFS and OS of the cohort were 30.5 and 39.13 months respectively. Only local therapy receipt was associated with improved EFS (multivariable HR:0.013; 95%CI:0.001–0.158; p &lt; 0.001) and OS (multivariable HR:0.028; 95 % CI:0.003–0.272; p = 0.002). Patients receiving radiotherapy alone had inferior OS compared to those receiving surgery alone. (HR: 9.22; 95 % CI: 1.12–76.31; p = 0.039)</div></div><div><h3>Conclusion</h3><div>ES-HF is a rare ES subsite. Although indolent, metastases are common at presentation. Meticulous local control can improve survival in both localized and metastatic disease for this subsite.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 24-32"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143683014","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cefiderocol for severe bloodstream infection by multidrug-resistant Klebsiella pneumoniae in a pediatric patient with acute myeloid leukemia 头孢地罗治疗急性髓性白血病患儿多重耐药肺炎克雷伯菌严重血流感染
Pediatric Hematology Oncology Journal Pub Date : 2025-03-01 DOI: 10.1016/j.phoj.2025.02.003
Francesco De Leonardis , Francesca Marasciulo , Roberta Koronica , Mariachiara Servedio , Enza Pentassuglia , Vittorio Greco Miani , Lidia Dalfino , Stefania Stolfa , Nicola Santoro
{"title":"Cefiderocol for severe bloodstream infection by multidrug-resistant Klebsiella pneumoniae in a pediatric patient with acute myeloid leukemia","authors":"Francesco De Leonardis ,&nbsp;Francesca Marasciulo ,&nbsp;Roberta Koronica ,&nbsp;Mariachiara Servedio ,&nbsp;Enza Pentassuglia ,&nbsp;Vittorio Greco Miani ,&nbsp;Lidia Dalfino ,&nbsp;Stefania Stolfa ,&nbsp;Nicola Santoro","doi":"10.1016/j.phoj.2025.02.003","DOIUrl":"10.1016/j.phoj.2025.02.003","url":null,"abstract":"","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 57-58"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143869653","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Challenges faced in screening for thalassemia and other hemoglobinopathies in a North Indian population 北印度人口地中海贫血和其他血红蛋白病筛查面临的挑战
Pediatric Hematology Oncology Journal Pub Date : 2025-03-01 DOI: 10.1016/j.phoj.2025.02.005
Manisha Kumar, Vani Kargwal, Rajeev Goel, Ekta Debnath, Seema Malhotra, Kirti Balyan, Mahrukh Zaidi, Reena Yadav
{"title":"Challenges faced in screening for thalassemia and other hemoglobinopathies in a North Indian population","authors":"Manisha Kumar,&nbsp;Vani Kargwal,&nbsp;Rajeev Goel,&nbsp;Ekta Debnath,&nbsp;Seema Malhotra,&nbsp;Kirti Balyan,&nbsp;Mahrukh Zaidi,&nbsp;Reena Yadav","doi":"10.1016/j.phoj.2025.02.005","DOIUrl":"10.1016/j.phoj.2025.02.005","url":null,"abstract":"<div><h3>Background</h3><div>Hemoglobinopathies are a common group of inherited disorders contributing significantly to the global healthcare burden, particularly in low and middle-income countries (LMICs) such as India.</div></div><div><h3>Objective</h3><div>This prospective cross-sectional study aimed to investigate the epidemiological, clinical, and genetic aspects of hemoglobinopathies in the antenatal population attending a tertiary care hospital in Delhi.</div></div><div><h3>Material and method</h3><div>A total of 7077 antenatal women were investigated over four years, revealing an overall prevalence of Beta Thalassemia Trait (BTT) of 4.55%. The screening algorithm involved a complete blood count (CBC) and High Performance Liquid Chromatography (HPLC) for all the women, followed by mutation testing using Multiplex amplification refractory mutation system - polymerase chain reaction (ARMS PCR) for four common mutations: IVS1-5 (G &gt; C), CD41/42 -TTCT, CD8/9 +G, and Del 619 bp. Multiplex ligation dependent probe amplification assay (MLPA), ARMS PCR and Sanger sequencing were used to identify other mutations. In cases where abnormal High Peformance Liquid Chromatography (HPLC) results were found, further testing of the husband was conducted to guide counselling and decisions regarding prenatal testing.</div></div><div><h3>Results</h3><div>IVS1-5 (G &gt; C) was identified as the most common mutation. Hemoglobin E (HbE) with CD26 (G &gt; A) exhibited the highest hemoglobin and HbA2 values amongst all mutations screened for. The study underscores the challenges of using RBC indices to diagnose BTT, due to marked overlap with Nutritional Deficiency Anemia (NDA). Similar issues were observed with HbD and HbE due to their relatively high MCV and MCH values. An algorithm for antenatal screening and diagnosis for LMIC is proposed but, given these caveats, its value remains to be proven.</div></div><div><h3>Conclusion</h3><div>The study provides data on the relative distribution of hemoglobinopathy mutations in the antenatal population in North India, and revealed the problems of detecting BTT or hemoglobinopathy in this population with a CBC due to the widespread prevalence of NDA. It emphasized the challenges of targeted screening strategies and genetic counselling to effectively reduce the prevalence of thalassemia in North India.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 59-65"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143869651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Current management of pulmonary relapse in Ewing sarcoma: A report from the Pediatric Surgical Oncology Research Collaborative 目前尤文氏肉瘤肺部复发的处理:一份来自儿科外科肿瘤研究协进会的报告
Pediatric Hematology Oncology Journal Pub Date : 2025-03-01 DOI: 10.1016/j.phoj.2024.11.105
Audra J. Reiter , Lynn Huang , Jennifer H. Aldrink , Brian T. Craig , Andrew M. Davidoff , Lindsay J. Talbot , Jordan Coggins , Jasmine Smith , Katherine C. Bergus , Taleen A. MacArthur , Stephanie F. Polites , Roshni Dasgupta , Chloe Boehmer , Joseph Brungardt , Marcus M. Malek , Hannah N. Rinehardt , Zachary J. Kastenberg , Cameron M. Arkin , Antoine Gourmel , Nelson Piche , Timothy B. Lautz
{"title":"Current management of pulmonary relapse in Ewing sarcoma: A report from the Pediatric Surgical Oncology Research Collaborative","authors":"Audra J. Reiter ,&nbsp;Lynn Huang ,&nbsp;Jennifer H. Aldrink ,&nbsp;Brian T. Craig ,&nbsp;Andrew M. Davidoff ,&nbsp;Lindsay J. Talbot ,&nbsp;Jordan Coggins ,&nbsp;Jasmine Smith ,&nbsp;Katherine C. Bergus ,&nbsp;Taleen A. MacArthur ,&nbsp;Stephanie F. Polites ,&nbsp;Roshni Dasgupta ,&nbsp;Chloe Boehmer ,&nbsp;Joseph Brungardt ,&nbsp;Marcus M. Malek ,&nbsp;Hannah N. Rinehardt ,&nbsp;Zachary J. Kastenberg ,&nbsp;Cameron M. Arkin ,&nbsp;Antoine Gourmel ,&nbsp;Nelson Piche ,&nbsp;Timothy B. Lautz","doi":"10.1016/j.phoj.2024.11.105","DOIUrl":"10.1016/j.phoj.2024.11.105","url":null,"abstract":"<div><h3>Background</h3><div>Relapse occurs in 30–40 % of patients with localized Ewing sarcoma (EWS). Our objective was to describe the current management and outcomes of patients with initially localized EWS who experience first pulmonary relapse.</div></div><div><h3>Methods</h3><div>This multi-center retrospective cohort study included patients ≤22 years old with initially localized EWS treated from 2007 to 2020 at 19 Pediatric Surgical Oncology Research Collaborative institutions, who developed pulmonary relapse. Kaplan-Meier analysis was performed.</div></div><div><h3>Results</h3><div>Thirty-three patients with initially localized EWS developed pulmonary relapse at a median age of 17 (IQR 14; 20) years. Eleven (33 %) patients also had extra-pulmonary metastases (EPM) at relapse. Among the 22 (67 %) patients with pulmonary-only relapse, 10 (45 %) had solitary pulmonary nodules. Pulmonary metastasectomy was performed in 8/10 (80 %) patients with solitary pulmonary-only metastases, 5/12 (42 %) patients with multiple pulmonary-only metastases, and 2/11 (18 %) patients who also had EPM. Whole lung irradiation was administered in 7/10 (70 %) with solitary pulmonary-only metastases, 7/12 (58 %) with multiple pulmonary-only metastases, and 2/11 (18 %) with EPM. Rates of further pulmonary relapse/progression were similar between groups (p = 0.97). In Kaplan-Meier analysis, 3-year overall survival was 73 % with solitary pulmonary-only metastases, 40 % with multiple pulmonary-only metastases, and 23 % with EPM (p = 0.097).</div></div><div><h3>Conclusions</h3><div>While survival for patients with relapsed EWS is poor, the subset of patients with solitary relapse confined to the lung are often good candidates for pulmonary metastasectomy and have a non-statistically significant trend towards improved survival outcomes.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 20-23"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143683013","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Prevalence of vitamin D deficiency in pediatric patients with sickle cell disease: A systematic review 儿童镰状细胞病患者维生素D缺乏症的患病率:一项系统综述
Pediatric Hematology Oncology Journal Pub Date : 2025-03-01 DOI: 10.1016/j.phoj.2025.02.001
Thiago de Souza Vilela , Mauro Fisberg , Gerson Ferrari , Josefina Aparecida Pellegrini Braga
{"title":"Prevalence of vitamin D deficiency in pediatric patients with sickle cell disease: A systematic review","authors":"Thiago de Souza Vilela ,&nbsp;Mauro Fisberg ,&nbsp;Gerson Ferrari ,&nbsp;Josefina Aparecida Pellegrini Braga","doi":"10.1016/j.phoj.2025.02.001","DOIUrl":"10.1016/j.phoj.2025.02.001","url":null,"abstract":"<div><div>Hypovitaminosis D is significantly more prevalent in sickle cell disease (SCD) compared to healthy control patients. The absence of adequate vitamin D levels in patients with a chronic inflammatory condition may exacerbate inflammation, worsening the disease. Therefore, a systematic review was conducted analyzing articles from the Medline database of the National Institute of Medicine between April 2023 and November 2023. Inclusion criteria were observational studies in pediatric populations up to 20 years old, published in English. Intervention studies, clinical trials, abstracts, and reviews were excluded. The final number of selected articles was 18 of 85 listed. The overall frequency of vitamin D deficiency in the pediatric age group with SCD was 50.49 % (569 out of 1127), considering 20 ng/mL (50 nmol/L) as the cutoff for deficiency, the most commonly used reference level for vitamin D deficiency in the listed studies. The prevalence of vitamin D deficiency in this population appeared to be higher when compared to children and adolescents without SCD. Our review concluded that vitamin D deficiency is prevalent in the pediatric population with SCD, but future studies should confirm the implications of this finding.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 42-47"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143704653","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Thalassemia major due to compound heterozygous Codon 8/9 (+G) mutation with Dutch beta° thalassemia deletion 主要的地中海贫血是由于复合杂合密码子8/9 (+G)突变与荷兰β°地中海贫血缺失
Pediatric Hematology Oncology Journal Pub Date : 2025-03-01 DOI: 10.1016/j.phoj.2024.11.120
Dolat Singh Shekhawat , Tanuja Rajial , Siyaram Didel , Abhishek Purohit , Kuldeep Singh
{"title":"Thalassemia major due to compound heterozygous Codon 8/9 (+G) mutation with Dutch beta° thalassemia deletion","authors":"Dolat Singh Shekhawat ,&nbsp;Tanuja Rajial ,&nbsp;Siyaram Didel ,&nbsp;Abhishek Purohit ,&nbsp;Kuldeep Singh","doi":"10.1016/j.phoj.2024.11.120","DOIUrl":"10.1016/j.phoj.2024.11.120","url":null,"abstract":"","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 48-50"},"PeriodicalIF":0.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143869652","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Management outcomes of South African children diagnosed with neuroblastoma in adolescence 南非青少年被诊断为神经母细胞瘤儿童的治疗结果
Pediatric Hematology Oncology Journal Pub Date : 2025-01-02 DOI: 10.1016/j.phoj.2024.12.003
Van Heerden Jaques , Esterhuizen Tonya , Du Plessis Jan , Geel Jennifer , Büchner Ané , Van Zyl Anel , Hendricks Marc , Naidu Gita , van Emmenes Barry , Vaithilingum M , Kruger Mariana
{"title":"Management outcomes of South African children diagnosed with neuroblastoma in adolescence","authors":"Van Heerden Jaques ,&nbsp;Esterhuizen Tonya ,&nbsp;Du Plessis Jan ,&nbsp;Geel Jennifer ,&nbsp;Büchner Ané ,&nbsp;Van Zyl Anel ,&nbsp;Hendricks Marc ,&nbsp;Naidu Gita ,&nbsp;van Emmenes Barry ,&nbsp;Vaithilingum M ,&nbsp;Kruger Mariana","doi":"10.1016/j.phoj.2024.12.003","DOIUrl":"10.1016/j.phoj.2024.12.003","url":null,"abstract":"<div><h3>Background</h3><div>Neuroblastoma (NB) is an indolent disease in adolescents. Only 5 % of neuroblastoma diagnoses are made in adolescents and adults. Limited data exists on Africans of older age groups.</div></div><div><h3>Objectives</h3><div>We aimed to compare the characteristics and outcomes of South African children ≥10 years diagnosed with NB with international reports.</div></div><div><h3>Patients and methods</h3><div>Multicentric, retrospective data of South African patients (0–15 y) with NB diagnosed between January 2000 and December 2016 were analyzed. Clinical profiles and outcomes of adolescents (≥10 years) with NB were compared to those of the younger patients in our cohort. The outcomes of adolescents (≥10 years) with NB were compared with those reported in the global literature.</div></div><div><h3>Results</h3><div>Thirty adolescents with a male-to-female ratio of 1:0.6 were diagnosed with NB, constituting 6.5 % of all patients with NB. Metastatic disease at diagnosis was present in 24 (75 %), including lung metastases in 2 (6 %). Half of the patients presented with a raised lactate dehydrogenase (LDH) and/or ferritin and unfavorable pathology.</div><div>Post-induction remission rates were higher in the adolescent group (33 %) compared to the 18–59 months (24.8 %) and 5–9.9 years (29.7 %) cohorts but less than the &lt;18 months (47.2 %) cohort (p &lt; 0.001). The adolescent group had the poorest five-year overall survival (20.9 %) compared to the children &lt;18 months (39.0 %), 18–59 months (44.1 %), or 5–9.9 years old (63.5 %) (p &lt; 0.001).</div></div><div><h3>Conclusions</h3><div>Despite a favorable response rate to induction chemotherapy, the five-year overall survival of adolescents with NB is dismal in South Africa.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 9-16"},"PeriodicalIF":0.0,"publicationDate":"2025-01-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143164846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Global initiative for childhood cancer focused tumors in Indonesia: A single-center study 印度尼西亚儿童癌症肿瘤全球倡议:单中心研究
Pediatric Hematology Oncology Journal Pub Date : 2024-12-16 DOI: 10.1016/j.phoj.2024.12.002
Braghmandita Widya Indraswari , Saskia Mostert , Danardono , Bambang Ardianto , Eddy Supriyadi , Gertjan Kaspers , Mei Neni Sitaresmi
{"title":"Global initiative for childhood cancer focused tumors in Indonesia: A single-center study","authors":"Braghmandita Widya Indraswari ,&nbsp;Saskia Mostert ,&nbsp;Danardono ,&nbsp;Bambang Ardianto ,&nbsp;Eddy Supriyadi ,&nbsp;Gertjan Kaspers ,&nbsp;Mei Neni Sitaresmi","doi":"10.1016/j.phoj.2024.12.002","DOIUrl":"10.1016/j.phoj.2024.12.002","url":null,"abstract":"<div><h3>Background</h3><div>The WHO has launched a Global Initiative to achieve 60 % childhood cancer survival in low— and middle-income countries. Their initial focus is on six highly curable types of cancer: acute lymphoblastic leukemia (ALL), Burkitt lymphoma (BL), Hodgkin lymphoma (HL), retinoblastoma, Wilms tumor (WT), and low-grade glioma (LGG). This study, therefore, investigates treatment outcomes and survival of children with highly curable cancer types in Indonesia.</div></div><div><h3>Methods</h3><div>Medical records of children diagnosed with curable cancer types between 2011 and 2016 at a large hospital were retrospectively abstracted until 2019.</div></div><div><h3>Results</h3><div>Six hundred and forty-four children were diagnosed with curable cancers: ALL (491; 76 %), retinoblastoma (61; 9 %), WT (43; 7 %), non-Hodgkin lymphoma (NHL) (43; 7 %), and HL (6; 1 %). Due to limited diagnostic tests, NHL could not be subclassified. The male-to-female ratio was 1.37. The mean age at diagnosis was 5.8 ± 4.6 years. Most (379; 59 %) had insurance at diagnosis. Event-free survival for at two years after diagnosis was 36 %. Abandonment was the most common treatment failure (26 %). The 4-year predicted event-free survival was highest for ALL (27 %) and lowest for NHL (17 %). The Cox proportional hazard model showed that cancer type (P &lt; 0.001), age at diagnosis (P = 0.010), and health insurance coverage (P &lt; 0.001) were associated with predicted event-free survival.</div></div><div><h3>Conclusion</h3><div>The current observed event-free survival of highly curable childhood cancers in Indonesia is lower than other LMICs. The main reasons for treatment failure must be addressed to improve care and achieve the envisioned 60 % cure rate.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"10 1","pages":"Pages 1-8"},"PeriodicalIF":0.0,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143164845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Editorial Board Information 编委会信息
Pediatric Hematology Oncology Journal Pub Date : 2024-12-01 DOI: 10.1016/S2468-1245(24)00366-8
{"title":"Editorial Board Information","authors":"","doi":"10.1016/S2468-1245(24)00366-8","DOIUrl":"10.1016/S2468-1245(24)00366-8","url":null,"abstract":"","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Page ii"},"PeriodicalIF":0.0,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143148844","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Curative hematopoietic stem cell transplantation in a child with specific granule deficiency due to a homozygous SMARCD2 variant 为一名因同源 SMARCD2 变异而患有特异性颗粒缺乏症的儿童实施治愈性造血干细胞移植手术
Pediatric Hematology Oncology Journal Pub Date : 2024-11-09 DOI: 10.1016/j.phoj.2024.11.103
Janaki Menon , Revathi Raj , Shiny Padinjarayil Manakkad , Athulya Edavazhippurath , Priya Saravanan , Anjit Unnikrishnan , Ramya Uppuluri , Sheena Othayoth Kandy , Shammy Saphia , Kalpana George , Dhananjayan Dhanasooraj , Geeta Madathil Govindaraj
{"title":"Curative hematopoietic stem cell transplantation in a child with specific granule deficiency due to a homozygous SMARCD2 variant","authors":"Janaki Menon ,&nbsp;Revathi Raj ,&nbsp;Shiny Padinjarayil Manakkad ,&nbsp;Athulya Edavazhippurath ,&nbsp;Priya Saravanan ,&nbsp;Anjit Unnikrishnan ,&nbsp;Ramya Uppuluri ,&nbsp;Sheena Othayoth Kandy ,&nbsp;Shammy Saphia ,&nbsp;Kalpana George ,&nbsp;Dhananjayan Dhanasooraj ,&nbsp;Geeta Madathil Govindaraj","doi":"10.1016/j.phoj.2024.11.103","DOIUrl":"10.1016/j.phoj.2024.11.103","url":null,"abstract":"<div><h3>Background</h3><div>Children with recurrent, severe infections that respond poorly to treatment are likely to have an underlying inborn error of immunity.</div></div><div><h3>Case Report</h3><div>A three-year-old male child of non-consanguineous parentage presented with recurrent severe infections from the neonatal period, including abscesses, pneumonia, diarrhea, meningitis, cystitis, and pyelonephritis. The child had subtle dysmorphic features. The dihydro rhodamine assay was abnormal, and the peripheral smear showed hypogranular, hypolobated neutrophils. Clinical exome sequencing revealed a homozygous variant in the SMARCD2 gene, confirming the diagnosis of specific granule deficiency 2. The child underwent a haploidentical hematopoietic transplant and is asymptomatic five months post-transplant. Conditioning chemotherapy included rabbit anti-thymocyte globulin/treosulphan/thiotepa/fludarabine. The CD34 dose infused was 15 x 10<sup>6</sup>/kilogram recipient body weight. The infused product was TCR alpha/beta and CD19 depleted with preserved TCR gamma/delta cells.</div></div><div><h3>Conclusion</h3><div>This case demonstrates that although ultra-rare inborn errors of immunity are often diagnosed by next-generation sequencing, simple hematological and immunological tests provide valuable clues. Timely hematopoietic stem cell transplantation is curative.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 311-314"},"PeriodicalIF":0.0,"publicationDate":"2024-11-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142697912","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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