Pediatric Hematology Oncology Journal最新文献

Management outcomes of South African children diagnosed with neuroblastoma in adolescence

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 , Esterhuizen Tonya , Du Plessis Jan , Geel Jennifer , Büchner Ané , Van Zyl Anel , Hendricks Marc , Naidu Gita , van Emmenes Barry , Vaithilingum M , 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 <18 months (47.2 %) cohort (p < 0.001). The adolescent group had the poorest five-year overall survival (20.9 %) compared to the children <18 months (39.0 %), 18–59 months (44.1 %), or 5–9.9 years old (63.5 %) (p < 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

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 , Saskia Mostert , Danardono , Bambang Ardianto , Eddy Supriyadi , Gertjan Kaspers , 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 < 0.001), age at diagnosis (P = 0.010), and health insurance coverage (P < 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

Pediatric Hematology Oncology Journal Pub Date : 2024-12-01 DOI: 10.1016/S2468-1245(24)00366-8

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 , 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","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}

引用次数: 0

Signet cell carcinoma of the colon in an 11-year-old child: A case report and review of literature 一名 11 岁儿童的结肠信号细胞癌：病例报告和文献综述

Pediatric Hematology Oncology Journal Pub Date : 2024-10-31 DOI: 10.1016/j.phoj.2024.10.155

Pragya Singh , Lekshmi R , Sweety Gupta , Ravi Roushan Kumar , Shreyosi Mandal , Mridul Khanna , Satya Sree Balija , Ravi Hari Phulware , Manoj Gupta

{"title":"Signet cell carcinoma of the colon in an 11-year-old child: A case report and review of literature","authors":"Pragya Singh , Lekshmi R , Sweety Gupta , Ravi Roushan Kumar , Shreyosi Mandal , Mridul Khanna , Satya Sree Balija , Ravi Hari Phulware , Manoj Gupta","doi":"10.1016/j.phoj.2024.10.155","DOIUrl":"10.1016/j.phoj.2024.10.155","url":null,"abstract":"<div><h3>Background</h3><div>Colon cancer is uncommon in children. This article reports a case of signet ring cell carcinoma in an 11-year-old child who presented with acute intestinal obstruction.</div></div><div><h3>Case report</h3><div>An 11-year-old boy presented with progressive abdominal distension associated with constipation. The child underwent an abdominal X-ray in the erect position which revealed dilated bowel loops. Contrast enhanced computed tomography abdomen reported circumferential thickening involving 3.5 cm of the mid-transverse colon. The child underwent exploratory laparotomy which revealed mid transverse colon tumor. Tumor was resected with side-to-side anastomosis. Postoperative histopathology showed signet ring cell carcinoma. He was started on adjuvant chemotherapy.</div></div><div><h3>Conclusion</h3><div>Surgery followed by adjuvant chemotherapy is the modality of treatment colorectal carcinoma. There are no screening guidelines available for the pediatric age group, however early diagnosis through colonoscopy screening can be considered to improve the prognosis.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 307-310"},"PeriodicalIF":0.0,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142697911","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

Updates in the diagnosis and management of hemolytic anemias 溶血性贫血诊断和管理的最新进展

Pediatric Hematology Oncology Journal Pub Date : 2024-10-05 DOI: 10.1016/j.phoj.2024.09.006

Jagdish Chandra, Pooja Dewan

引用次数: 0

Meigs syndrome, pseudo-Meigs syndrome, or pseudo-pseudo Meigs syndrome? A case report 梅格斯综合征、假梅格斯综合征还是假-假梅格斯综合征？病例报告

Pediatric Hematology Oncology Journal Pub Date : 2024-09-24 DOI: 10.1016/j.phoj.2024.09.004

Azie Jumaatul Adawiyah Nabir , Chiew Yee Lau , Adilah W. Ab Rahim , Aliyyah Mohammad Khuzaini

{"title":"Meigs syndrome, pseudo-Meigs syndrome, or pseudo-pseudo Meigs syndrome? A case report","authors":"Azie Jumaatul Adawiyah Nabir , Chiew Yee Lau , Adilah W. Ab Rahim , Aliyyah Mohammad Khuzaini","doi":"10.1016/j.phoj.2024.09.004","DOIUrl":"10.1016/j.phoj.2024.09.004","url":null,"abstract":"<div><h3>Background</h3><div>Meigs syndrome, pseudo-Meigs syndrome, and pseudo-pseudo-Meigs syndrome showcase a diagnostic challenge in the management of complex pediatric cases.</div></div><div><h3>Case report</h3><div>We report a case of a 6-year-old girl who presented with progressive breathlessness, lethargy, and constipation for two weeks, associated with multiple episodes of joint pain and polymorphous rash. Imaging of the thorax, abdomen, and pelvis suggested bilateral pleural effusion, ascites, and an ovarian mass with abdominal involvement. The pleural and peritoneal fluids were exudative in nature. Unfortunately, she was not fit for a biopsy of the mass and was treated in the intensive care unit for one month. She was initiated on oral corticosteroids for suspicion of an underlying auto-inflammatory disease. However, subsequent investigations yielded inconclusive results. She gradually recovered and was well for a year. She presented again with constitutional symptoms and was diagnosed with diffuse large B-cell lymphoma (DLBCL). Thus, it was retrospectively apparent that this was a case of pseudo-Meigs syndrome.</div></div><div><h3>Conclusion</h3><div>We describe an unusual presentation of DLBCL with a temporary resolution of symptoms.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 289-293"},"PeriodicalIF":0.0,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142697908","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

Clinical outcomes of restrictive versus liberal blood transfusion strategies in critical care children: A systematic review and meta-analysis of randomized controlled trials 危重症儿童限制性输血策略与自由输血策略的临床结果：随机对照试验的系统回顾和荟萃分析

Pediatric Hematology Oncology Journal Pub Date : 2024-09-16 DOI: 10.1016/j.phoj.2024.09.005

Nabeel Al-Yateem , Fatma Refaat Ahmed , Seyed Aria Nejadghaderi

{"title":"Clinical outcomes of restrictive versus liberal blood transfusion strategies in critical care children: A systematic review and meta-analysis of randomized controlled trials","authors":"Nabeel Al-Yateem , Fatma Refaat Ahmed , Seyed Aria Nejadghaderi","doi":"10.1016/j.phoj.2024.09.005","DOIUrl":"10.1016/j.phoj.2024.09.005","url":null,"abstract":"<div><h3>Background</h3><div>The published experience with restrictive and liberal blood transfusion approaches in pediatric intensive care units (ICUs) is not extensive. We investigated the outcomes of restrictive compared to liberal transfusion strategies in critically ill children.</div></div><div><h3>Methods</h3><div>A search was conducted on PubMed, Embase, Scopus, and the Web of Science until February 16, 2024. The first 300 results from Google Scholar and records from the <span><span>clinicaltrials.gov</span><svg><path></path></svg></span> registry were manually screened. Backward and forward citation searches were also performed. We included randomized controlled trials examining outcome measures of children aged <18 years admitted to the ICUs who received restrictive methods for blood transfusion compared with the liberal one.</div></div><div><h3>Results</h3><div>Ten studies were included, with a pooled sample size of 2736. Half (50.8 %) were males; age ranged from 2.5 days to 73.7 months. The restrictive transfusion strategy significantly decreased the risk of nosocomial infections (risk ratio: 0.64; 95 % confidence interval: 0.42, 0.96) and also reduced hemoglobin levels (standardized mean difference: −2.92; 95 % CI: −4.48, −1.35), while there were no significant changes between these blood transfusion strategies in terms of death, safety or adverse events, duration of hospital and ICU stay, and other measures, such as length of hospital and ICU stay, hematocrit, serum lactate, and serum ferritin levels. Quality assessment indicated that most studies had some concerns (n = 7), and others had high (n = 2) or low (n = 1) risk of bias.</div></div><div><h3>Conclusions</h3><div>Restrictive transfusion strategy is safe and effective for critically ill children.</div></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 297-306"},"PeriodicalIF":0.0,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142697909","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

Human adenovirus infection with secondary hemophagocytic lymphohistiocytosis in immunocompromised children in clusters from South India 南印度免疫力低下儿童集群感染人类腺病毒并继发嗜血细胞淋巴组织细胞增多症

Pediatric Hematology Oncology Journal Pub Date : 2024-09-12 DOI: 10.1016/j.phoj.2024.09.001

Sudeep Gaddam, Sivaram Ragavan, Dhaarani Jayaraman, Padmasani Venkat Ramanan, Padma Srikanth, Shuba Sankaranarayanan, Latha M. Sneha, Julius Xavier Scott

引用次数: 0

Juvenile myelomonocytic leukemia masquerading as Langerhans cell histiocytosis: An immuno-morphologic dilemma 伪装成朗格汉斯细胞组织细胞增生症的幼年髓单核细胞白血病：免疫形态学难题

Pediatric Hematology Oncology Journal Pub Date : 2024-09-10 DOI: 10.1016/j.phoj.2024.09.002

Kala Gnanasekaran Kiruthiga , Sarita Verma Kokane , Kannan Subramanian , Avinash Pradhan , Ravi Godbole

{"title":"Juvenile myelomonocytic leukemia masquerading as Langerhans cell histiocytosis: An immuno-morphologic dilemma","authors":"Kala Gnanasekaran Kiruthiga , Sarita Verma Kokane , Kannan Subramanian , Avinash Pradhan , Ravi Godbole","doi":"10.1016/j.phoj.2024.09.002","DOIUrl":"10.1016/j.phoj.2024.09.002","url":null,"abstract":"<div><h3>Background</h3><p>Non-neoplastic, reactive proliferation of Langerhans cells is observed in leukemias, pseudo-lymphomas, carcinomas, etc. Juvenile myelomonocytic leukemia (JMML) is a rare myelodysplastic/myeloproliferative neoplasm with a few overlapping clinical features to Langerhans cell histiocytosis (LCH). JMML involving the lymph nodes may show the proliferation of Langerhans cells.</p></div><div><h3>Case report</h3><p>A two year old boy presented with fever, hepatosplenomegaly and elevated total leukocyte count with monocytosis. Bone marrow revealed dysplasia in erythroids and myeloids. Axillary lymph node showed sheets of macrophages in sinuses and paracortical areas (S100, CD68 and CD 1a positive) admixed with eosinophils, mimicking LCH. Molecular analysis revealed somatic heterozygous, missense mutation in PTPN11 exon 3, a known pathogenic hot spot mutation in JMML.</p></div><div><h3>Conclusion</h3><p>The treatment and prognosis of JMML and LCH are different. JMML may have a clinical and morphological overlap with LCH and hence can be misdiagnosed. This paper highlights the similarities and differences between the two diseases with a case illustration.</p></div>","PeriodicalId":101004,"journal":{"name":"Pediatric Hematology Oncology Journal","volume":"9 4","pages":"Pages 267-270"},"PeriodicalIF":0.0,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2468124524000767/pdfft?md5=43f40bbf610c4051192f16e37e3244aa&pid=1-s2.0-S2468124524000767-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142243679","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