ReviewPaediatric drugs2010
Juvenile myelomonocytic leukemia: epidemiology, etiopathogenesis, diagnosis, and management considerations.
Review in Paediatric drugs, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
11 citing papers in PubMed, 26 citations in OpenAlex.
- Juvenile Myelomonocytic Leukemia in a Child: A Case Report of Palliative Chemotherapy and Literature Review Applied to Limited Resources Centers.Case reports in hematology · 2022Article
- Cutaneous Findings in Neurofibromatosis Type 1.Cancers · 2021Review
- Article
- [Clinical and genetic analyses of juvenile myelomonocytic leukemia].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2019Article
- MiRNA Dysregulation in Childhood Hematological Cancer.International journal of molecular sciences · 2018Review
- VAV1-Cre mediated hematopoietic deletion of CBL and CBL-B leads to JMML-like aggressive early-neonatal myeloproliferative disease.Oncotarget · 2016Article
- Management of Two Juvenile Myelomonocytic Leukemia Patients According to Clinical and Genetic Features.Turkish journal of haematology : official journal of Turkish Society of Haematology · 2015Article
- A RASopathy gene commonly mutated in cancer: the neurofibromatosis type 1 tumour suppressor.Nature reviews. Cancer · 2015Review
- Hematopoetic stem cell transplantation in children.Turk pediatri arsivi · 2014Review
- Neurofibromatosis type 1-associated tumours: their somatic mutational spectrum and pathogenesis.Human genomics · 2011Review
- Juvenile myelomonocytic leukemia characterized by cutaneous lesion containing Langerhans cell histiocytosis-like cells.International journal of hematology · 2011Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
3 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Juvenile myelomonocytic leukemia (JMML) is a rare hematopoietic malignancy of early childhood with features characteristic of both myelodysplastic and myeloproliferative disorders. Recent studies clearly show that the deregulated activation of the RAS signaling pathway plays a central role in the pathogenesis of JMML. Somatic defects in either RAS, PTPN11 or NF1 genes involved in this pathway are detected in 70-80% of JMML patients, allowing a molecular diagnosis to be made in the majority of cases. Patients with JMML respond poorly to chemotherapy, and the probability of survival without allogeneic hematopoietic stem cell transplantation (HSCT) is less than 10%. Recent studies show that the event-free survival after HSCT is between 24 and 54%, with no difference between transplants using matched family donors and those using unrelated donors. The use of therapies such as intensive chemotherapy and splenectomy prior to HSCT does not improve the outcome. The relapse rate following HSCT is over 30%, which is unacceptably high. Cumulative evidence suggests that a graft-versus-leukemia effect occurs in JMML. Donor leukocyte infusion is not usually successful in JMML, but the outcome of second HSCT is generally favorable. Based on recent advances in the understanding of the pathogenesis of JMML, the development of novel targeted therapies, which might improve the outcome of patients, is keenly awaited.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.