Evidence map›Paper›PMID 27313483›Full record

ArticleJournal of blood medicine2016

Development of paroxysmal nocturnal hemoglobinuria in CALR-positive myeloproliferative neoplasm.

Yarden S Fraiman, Nathan Cuka, Denise Batista, Milena Vuica-Ross, Alison R Moliterno

Open access · goldAbstract read
In one paragraph

Article in Journal of blood medicine, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
1.2field-weighted citation impact, top 21% 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

17 citing papers in PubMed, 22 citations in OpenAlex.

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  15. Paroxysmal nocturnal hemoglobinuria without GPI-anchor deficiency.The Journal of clinical investigation · 2019
    Article
  16. Article
  17. Analysis ofExperimental hematology & oncology · 2019
    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

5 authors at 3 institutions in 1 country.

Yarden S FraimanDepartment of Pediatrics, Harvard Medical School, Boston, MA, USA; Department of Pediatrics, Boston University School of Medicine, Boston, MA, USA.
Nathan CukaDepartment of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Denise BatistaDepartment of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Milena Vuica-RossDepartment of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Alison R MoliternoDivision of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Johns Hopkins Medicine · USJohns Hopkins University · USHarvard University · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH), a disease characterized by intravascular hemolysis, thrombosis, and bone marrow failure, is associated with mutations in the PIG-A gene, resulting in a deficiency of glycosylphosphatidylinositol-anchored proteins. Many hypotheses have been posed as to whether PNH and PIG-A mutations result in an intrinsic survival benefit of CD55(-)/CD59(-) cells or an extrinsic permissive environment that allows for their clonal expansion within the bone marrow compartment. Recent data have identified the concurrence of PIG-A mutations with additional genetic mutations associated with myeloproliferative disorders, suggesting that some presentations of PNH are the result of a stepwise progression of genetic mutations similar to other myelodysplastic or myeloproliferative syndromes. We report for the first time in the literature the development of clinically significant PNH in a patient with JAK2V617F-negative, CALR-positive essential thrombocythemia, providing further support to the hypothesis that the development of PNH is associated with the accumulation of multiple genetic mutations that create an intrinsic survival benefit for clonal expansion. This case study additionally highlights the utility of genomic testing in diagnosis and the understanding of disease progression in the clinical setting.

Indexed as

calreticulinmyelofibrosisPIGA deletionSNP array

Identifiers

PMID27313483
PMCPMC4892839
OpenAlexW2423732094

What OpenQuestion holds

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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.