Evidence map›Paper›PMID 28395106›Full record

ArticleAmerican journal of clinical pathology2017

Primary/Congenital Immunodeficiency: 2015 SH/EAHP Workshop Report-Part 5.

Dita Gratzinger, Elaine S Jaffe, Amy Chadburn, John K C Chan, Daphne de Jong, John R Goodlad, Jonathan Said, Yasodha Natkunam

Open access · bronzeAbstract read
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Article in American journal of clinical pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.

0numbers the graph read from it
0cells of the map it votes in
10citing papers in PubMed
1.1field-weighted citation impact, top 22% 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

10 citing papers in PubMed, 19 citations in OpenAlex.

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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

8 authors at 7 institutions in 4 countries.

Dita GratzingerFrom the Stanford University School of Medicine, Stanford, CA.
Elaine S JaffeNational Institutes of Health, Bethesda, MD.
Amy ChadburnWeill Medical College of Cornell University, New York, NY.
John K C ChanQueen Elizabeth Hospital, Kowloon, Hong Kong.
Daphne de JongVU University Medical Center, Amsterdam, the Netherlands.
John R GoodladUniversity of Leeds, United Kingdom.
Jonathan SaidUniversity of California Los Angeles Medical Center, Los Angeles.
Yasodha NatkunamFrom the Stanford University School of Medicine, Stanford, CA.
Stanford University · USAmsterdam UMC Location VUmc · NLCornell University · USLos Angeles Medical Center · USNational Institutes of Health · USQueen Elizabeth Hospital · CNUniversity of Leeds · GB

Funding

Hematopathology DiagnosisZIABC011070 · NCI · DIVISION OF BASIC SCIENCES - NCI · PI JAFFE, ELAINE · 2009 to 2025
$14.5M
6 · The paper itself

Abstract

objectivesThe 2015 Workshop of the Society for Hematopathology/European Association for Haematopathology aimed to review primary immunodeficiency and related lymphoproliferations.

methodsPrimary immunodeficiencies were divided into immune dysregulation, DNA repair defects, low immunoglobulins, and combined immunodeficiencies.

resultsAutoimmune lymphoproliferative syndrome (ALPS) is a prototypical immune dysregulation-type immunodeficiency, with defects in T-cell signaling or apoptosis, expansion of T-cell subsets, and predisposition to hemophagocytic lymphohistiocytosis. DNA repair defects directly predispose to malignancy. Low immunoglobulin immunodeficiencies such as common variable immunodeficiency (CVID) have underlying T-cell repertoire abnormalities predisposing to autoimmunity and B-cell lymphoproliferations. The full spectrum of B-cell lymphoproliferative disorders occurs in primary immunodeficiency.

conclusionsLymphoproliferations in primary immunodeficiency mirror those in other immunodeficiency settings, with monomorphic B- and sometimes T lymphoproliferative disorders enriched in DNA repair defects. Distinctive T-cell subset expansions in ALPS, CVID, and related entities can mimic lymphoma, and recognition of double-negative T-cell or cytotoxic T-cell expansions is key to avoid overdiagnosis.

Indexed as

EducationFemaleHumansImmunologic Deficiency SyndromesLymphoproliferative DisordersMaleAutoimmune lymphoproliferative syndromeCommon variable immunodeficiencyDouble negative T cellsPrimary immunodeficiencyT-cell lymphoma

Identifiers

PMID28395106
PMCPMC6248572
OpenAlexW2589596420

What OpenQuestion holds

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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.