Evidence map›Paper›PMID 39020342›Full record

ArticleOrphanet journal of rare diseases2024

Diagnostic landscape of first-time cytometric screening for paroxysmal nocturnal hemoglobinuria in Poland in 2013-2022.

Justyna Spychalska, Magdalena Duńska, Anna Myślińska, Monika Majewska-Wierzbicka, Edyta Klimczak-Jajor, Eliza Głodkowska-Mrówka

Abstract read
In one paragraph

Article in Orphanet journal of rare diseases, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed, 1 pooled it
–field-weighted citation impact
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

2 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. 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.

Justyna SpychalskaDepartment of Hematological and Transfusion Immunology, Institute of Hematology and Transfusion Medicine, Chocimska 5, Warsaw, 00-791, Poland.
Magdalena DuńskaDepartment of Hematological and Transfusion Immunology, Institute of Hematology and Transfusion Medicine, Chocimska 5, Warsaw, 00-791, Poland.
Anna MyślińskaDepartment of Hematological and Transfusion Immunology, Institute of Hematology and Transfusion Medicine, Chocimska 5, Warsaw, 00-791, Poland.
Monika Majewska-WierzbickaDepartment of Hematological and Transfusion Immunology, Institute of Hematology and Transfusion Medicine, Chocimska 5, Warsaw, 00-791, Poland.
Edyta Klimczak-JajorDepartment of Hematological and Transfusion Immunology, Institute of Hematology and Transfusion Medicine, Chocimska 5, Warsaw, 00-791, Poland.
Eliza Głodkowska-MrówkaDepartment of Hematological and Transfusion Immunology, Institute of Hematology and Transfusion Medicine, Chocimska 5, Warsaw, 00-791, Poland. eglodkowskam@ihit.waw.pl.ORCID 0000-0002-5865-8425

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundParoxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disorder characterized by PIG-A mutations, leading to glycophosphatidylinositol (GPI)-anchored proteins deficiency that triggers hemolysis - a hallmark of the disease. PNH diagnostics is based on high-sensitivity multicolor flow cytometry (MFC), enabling to detect even small populations of PNH cells. In this single-center, retrospective study, we aimed to characterize a cohort of PNH clone-positive patients first time screened from January 1st, 2013 until December 31st, 2022 with MFC according to International Clinical Cytometry Society PNH Consensus Guidelines.

resultsOut of 2790 first-time screened individuals, the presence of PNH clone in neutrophils was detected in 322 patients, including 49 children and 273 adults. Annual incidence was stable at a median of 31 patients (14 and 19 with clone sizes ≤ 1% and > 1%, respectively), with a decline in number of patients with clone sizes > 1% observed in 2020, potentially influenced by the COVID-19 pandemic. The most common screening indications were aplastic anemia and other cytopenias.

conclusionsA significant underrepresentation of hemolytic patients was observed as compared to the published cohorts suggesting that these patients are missed in diagnostic process and classic PNH remains underdiagnosed in Poland.

Indexed as

Flow CytometryHemoglobinuria, ParoxysmalAdolescentAdultAgedChildChild, PreschoolCOVID-19FemaleHumansInfantMaleMiddle AgedPolandRetrospective StudiesYoung Adult

Identifiers

PMID39020342
PMCPMC11256427

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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.