Evidence map›Paper›PMID 30679326›Full record

SynthesisHaematologica2019

Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age: a systematic review.

Jean-Christophe Ianotto, Natalia Curto-Garcia, Marie Lauermanova, Deepti Radia, Jean-Jacques Kiladjian, Claire N Harrison

Open access · goldAbstract readSystematic Review
In one paragraph

Synthesis in Haematologica, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.

0numbers the graph read from it
0cells of the map it votes in
39citing papers in PubMed
4.7field-weighted citation impact, top 5% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

39 citing papers in PubMed, 72 citations in OpenAlex.

  1. Article
  2. Review
  3. [Progress in the study of polycythemia vera in children].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2026
    Review
  4. Polycythemia vera as a cause of systemic hypertension.Pediatric nephrology (Berlin, Germany) · 2026
    Article
  5. Epigenetic alterations in Myeloid Malignancies.Advances in experimental medicine and biology · 2026
    Review
  6. Article
  7. Etiology, Diagnosis, and Genetic Analysis of Polycythemia in Pediatric Population.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2025
    Article
  8. Article
  9. [Advances in the diagnosis and treatment of thrombocytosis in children].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2025
    Review
  10. Article
  11. Article
  12. Review
  13. Article
  14. Article
  15. Article
  16. Article
  17. Review
  18. Article
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 3 institutions in 3 countries.

Jean-Christophe IanottoDepartment of Haematology, Guy's and St Thomas' NHS Trust, London, UK.
Natalia Curto-GarciaDepartment of Haematology, Guy's and St Thomas' NHS Trust, London, UK.
Marie LauermanovaDepartment of Haematology, Guy's and St Thomas' NHS Trust, London, UK.
Deepti RadiaDepartment of Haematology, Guy's and St Thomas' NHS Trust, London, UK.
Jean-Jacques KiladjianCentre d'Investigation Clinique, Hôpital St Louis, Paris, France.
Claire N HarrisonDepartment of Haematology, Guy's and St Thomas' NHS Trust, London, UK claire.harrison@gstt.nhs.uk.
Guy's and St Thomas' NHS Foundation Trust · GBHôpital Saint-Louis · FRInstitute of Haematology and Blood Transfusion · CZ

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Although it is well known that myeloproliferative neoplasms occur in younger patients, few large cohorts of such patients have been reported. Thus, our knowledge about circumstances of diagnosis, outcome and treatment is limited, especially for children and young adults. We therefore performed a systematic review of cases, published since 2005, concerning patients aged below 20 years at the time of diagnosis of essential thrombocythemia or polycythemia vera. We identified 396 cases of essential thrombocythemia and 75 of polycythemia vera. The median age at diagnosis was 9.3 and 12 years, respectively, and females constituted 57.6% and 45% of the groups, respectively. Half of the patients were asymptomatic at diagnosis. The proportion of so-called triple negativity was high: 57% in essential thrombocythemia and 73% in polycythemia vera. The incidence of thrombosis during the follow-up was 9.3% in patients with polycythemia vera and less, 3.8%, in those with essential thrombocythemia. Venous events were predominant (84.2%), with hemorrhagic episodes being rarer (<5%). The risk of evolution also seemed low (2% to myelofibrosis and no reports of acute leukemia), but the median follow-up was only 50 months. Survival curves were not available. Half of the patients received an antithrombotic drug and 40.5% received a cytoreductive drug. All data should be analyzed with care because of the proportion of missing data (10.7% to 74.7%). This review highlights interesting points concerning this population of young patients with myeloproliferative neoplasms, including that such patients were identified as negative for all common driver mutations, but also shows the need for larger contemporary cohorts with longer follow-up to assess the true prognosis of these patients.

Indexed as

AdolescentAsymptomatic DiseasesChildCytotoxinsEarly DiagnosisFibrinolytic AgentsGene ExpressionHemorrhageHumansJanus Kinase 1Janus Kinase 2MutationPolycythemia VeraPrognosisSplenomegalyThrombocythemia, EssentialCytotoxinsFibrinolytic AgentsJAK1 protein, humanJAK2 protein, humanJanus Kinase 1Janus Kinase 2

Identifiers

PMID30679326
PMCPMC6669170
OpenAlexW2911450427

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

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.