ReviewHematology. American Society of Hematology. Education Program2016
Myelodysplastic and myeloproliferative disorders of childhood.
Review in Hematology. American Society of Hematology. Education Program, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 94 citations in OpenAlex.
- Aberrant Expression ofBioMed research international · 2019Trial
- Clinical characteristics and long-term treatment outcomes in childhood myelodysplastic neoplasm: a nationwide population-based study in Korea.BMC cancer · 2026Article
- Decitabine monotherapy for pediatric myelodysplastic syndrome: A case report.Experimental and therapeutic medicine · 2026Article
- Cytogenetic Abnormalities in Pediatric Myelodysplastic Syndrome: Insights on the Disease Biology and Impact on Leukemic Evolution.Biomedicines · 2025Article
- The role of genetic factors in pediatric myelodysplastic syndromes with different outcomes.BMC pediatrics · 2024Article
- Association between Leukemic Evolution and Uncommon Chromosomal Alterations in Pediatric Myelodysplastic Syndrome.Mediterranean journal of hematology and infectious diseases · 2024Article
- A Rare Concurrence of Myelodysplastic Neoplasia and Tetrasomy 8 in a 3-Year-Old Bahraini Male.Cureus · 2023Article
- A Novel Constitutional t(3;8)(p26;q21) andJournal of clinical medicine · 2023Article
- An Updated Overview of the Role of CYP450 during Xenobiotic Metabolization in Regulating the Acute Myeloid Leukemia Microenvironment.International journal of molecular sciences · 2023Review
- Childhood cancer and hematological disorders negatively affect spermatogonial quantity at diagnosis: a retrospective study of a male fertility preservation cohort.Human reproduction (Oxford, England) · 2023Article
- Syndromes predisposing to leukemia are a major cause of inherited cytopenias in children.Haematologica · 2022Article
- The effect of decitabine-combined minimally myelosuppressive regimen bridged allo-HSCT on the outcomes of pediatric MDS from 10 years' experience of a single center.BMC pediatrics · 2022Article
- Diagnostic Value of a Protocolized In-Depth Evaluation of Pediatric Bone Marrow Failure: A Multi-Center Prospective Cohort Study.Frontiers in immunology · 2022Article
- Chronic Granulomatous Disease and Myelodysplastic Syndrome in a Patient with a Novel Mutation inGenes · 2021Article
- Inherited deficiency of stress granule ZNFX1 in patients with monocytosis and mycobacterial disease.Proceedings of the National Academy of Sciences of the United States of America · 2021Article
- Definition, Epidemiology, Pathophysiology, and Essential Criteria for Diagnosis of Pediatric Chronic Myeloid Leukemia.Cancers · 2021Review
- Article
- Improved outcomes of allogeneic hematopoietic stem cell transplantation including haploidentical transplantation for childhood myelodysplastic syndrome.Bone marrow transplantation · 2020Article
- Expression Profiles of DNA Methylation and Demethylation Machinery Components in Pediatric Myelodysplastic Syndrome: Clinical Implications.Cancer management and research · 2020Article
- The zebrafish: A fintastic model for hematopoietic development and disease.Wiley interdisciplinary reviews. Developmental biology · 2018Review
Corrections and comments
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Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions. RCC is often hypoplastic and may respond to immunosuppressive therapy. In case of immunosuppressive therapy failure, hypercellular RCC, or RCC with monosomy 7, hematopoietic stem cell transplantation (HSCT) using reduced-intensity conditioning regimens is indicated. Almost all patients with refractory anemia with excess blasts are candidates for HSCT; children age 12 years or older have a higher risk of treatment-related death, and the conditioning regimens should be adjusted accordingly. Unraveling the genetics of JMML has demonstrated that JMML in patients with germ line PTPN11 and CBL mutations often regresses spontaneously, and therapy is seldom indicated. Conversely, patients with JMML and neurofibromatosis type 1, somatic PTPN11, KRAS, and most of those with NRAS mutations have a rapidly progressive disease, and early HSCT is indicated. The risk of relapse after HSCT is high, and prophylaxis for graft-versus-host disease and monitoring should be adapted to this risk.
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