Evidence map›Paper›PMID 36716137›Full record

SynthesisBlood advances2023

Clinical and epidemiological features of paroxysmal cold hemoglobinuria: a systematic review.

Jeremy W Jacobs, Cristina A Figueroa Villalba, Garrett S Booth, Jennifer S Woo, Laura D Stephens, Brian D Adkins

Open access · goldAbstract readSystematic Review
In one paragraph

Synthesis in Blood advances, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
22citing papers in PubMed, 1 pooled it
10.2field-weighted citation impact, top 1% 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

22 citing papers in PubMed, 1 synthesis or guideline pooled it, 35 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Article
  4. Article
  5. Article
  6. Article
  7. Article
  8. Review
  9. Petechiae and Limb Weakness in a 12-Year-Old Boy.Dermatology practical & conceptual · 2025
    Article
  10. Review
  11. Article
  12. Review
  13. Autoimmune haemolytic anaemias.Nature reviews. Disease primers · 2024
    Review
  14. Article
  15. Article
  16. Article
  17. Review
  18. Review
  19. Article
  20. Review
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 5 institutions in 1 country.

Jeremy W JacobsDepartment of Laboratory Medicine, Yale School of Medicine, New Haven, CT.ORCID 0000-0002-5719-9685
Cristina A Figueroa VillalbaDepartment of Laboratory Medicine, Yale School of Medicine, New Haven, CT.
Garrett S BoothDepartment of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, TN.ORCID 0000-0002-8422-9480
Jennifer S WooDepartment of Pathology, City of Hope National Medical Center, Irvine, CA.
Laura D StephensDepartment of Pathology, University of California San Diego, La Jolla, CA.
Brian D AdkinsDivision of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern, Dallas, TX.ORCID 0000-0002-9480-2762
Yale University · USCity Of Hope National Medical Center · USThe University of Texas Southwestern Medical Center · USUniversity of California San Diego · USVanderbilt University Medical Center · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Paroxysmal cold hemoglobinuria (PCH) is a rare autoimmune hemolytic anemia often overlooked as a potential etiology of hemolysis and is challenging to diagnose because of the complicated testing methods required. We performed a systematic review of all reported cases to better assess the clinical, immunohematologic, and therapeutic characteristics of PCH. We systematically analyzed PubMed, Medline, and EMBASE to identify all cases of PCH confirmed by Donath-Landsteiner (DL) testing. Three authors independently screened articles for inclusion, and systematically extracted epidemiologic, clinical, laboratory, treatment, and outcomes data. Discrepancies were adjudicated by a fourth author. We identified 230 cases, with median presentation hemoglobin of 6.5 g/dL and nadir of 5.5 g/dL. The most common direct antiglobulin test (DAT) result was the presence of complement and absence of immunoglobulin G (IgG) bound to red blood cells, although other findings were observed in one-third of cases. DL antibody class and specificity were reported for 71 patients, of which 83.1% were IgG anti-P. The use of corticosteroids is common, although we found no significant difference in the length of hospitalization for patients with and without steroid therapy. Recent reports have highlighted the use of complement inhibitors. Among patients with follow-up, 99% (213 of 216) were alive at the time of reporting. To our knowledge, this represents the largest compilation of PCH cases to date. We discovered that contemporary PCH most commonly occurs in children with a preceding viral infection, corticosteroid use is frequent (but potentially ineffective), and DAT results are more disparate than traditionally reported.

Indexed as

Anemia, Hemolytic, AutoimmuneHemoglobinuria, ParoxysmalAdrenal Cortex HormonesChildErythrocytesHumansImmunoglobulin GAdrenal Cortex HormonesImmunoglobulin G

Identifiers

PMID36716137
PMCPMC10248093
OpenAlexW4318486271

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
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.