Evidence map›Paper›PMID 20538792›Full record

GuidelineBlood2010

Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop.

Joao B Oliveira, Jack J Bleesing, Umberto Dianzani, Thomas A Fleisher, Elaine S Jaffe, Michael J Lenardo, Frederic Rieux-Laucat, Richard M Siegel, Helen C Su, David T Teachey and 1 more

Open access · bronzeAbstract readConsensus Development Conference, NIHPractice Guideline
In one paragraph

Guideline in Blood, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 196 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
196citing papers in PubMed, 4 pooled it
13.2field-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

196 citing papers in PubMed, 4 syntheses or guidelines pooled it, 481 citations in OpenAlex.

  1. Red flags to suspect inborn errors of immunity in patients with autoimmune diseasesBiomedica : revista del Instituto Nacional de Salud · 2024
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136 more citing papers are in PubMed but not listed here.

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

11 authors at 8 institutions in 4 countries.

Joao B OliveiraDepartment of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA. oliveirajb@cc.nih.gov
Jack J Bleesing
Umberto Dianzani
Thomas A Fleisher
Elaine S Jaffe
Michael J Lenardo
Frederic Rieux-Laucat
Richard M Siegel
Helen C Su
David T Teachey
V Koneti Rao
National Institutes of Health · USNational Institutes of Health Clinical Center · USCancer Institute (WIA) · INChildren's Hospital of Philadelphia · USCincinnati Children's Hospital Medical Center · USNational Institute of Arthritis and Musculoskeletal and Skin Diseases · USUniversità degli Studi del Piemonte Orientale “Amedeo Avogadro” · ITUniversité Paris Cité · FR

Funding

Defining New Human Immunodeficiency and Immunodysregulation DisordersZIAAI001059 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI SU, HELEN CHUN-HUI · 2009 to 2025
$20.5M
Molecular Mechanisms Of The Autoimmune Lymphoproliferative Syndrome And Other Immunoregulatory DisordersZIAAI000769 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI SU, HELEN · 2009 to 2024
$16.7M
Clinical, Immunological and Genetic Analyses of ALPS and Related Immunedysregulatory DisordersZIAAI000732 · NIAID · NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES · PI RAO, V KONETI · 2009 to 2025
$10.5M
TNF-receptors and programmed cell death in regulation of autoimmunityZIAAR041133 · NIAMS · NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES · PI SIEGEL, RICHARD M · 2009 to 2019
$9.3M
Immune Evaluation in Patients with Autoimmune Lymphoproliferative SyndromeZIACL010287 · CLC · CLINICAL CENTER · PI FLEISHER, THOMAS A · 2009 to 2015
$0k
Intramural NIH HHS
6 · The paper itself

Abstract

Lymphadenopathy in children for which no infectious or malignant cause can be ascertained constitutes a challenging diagnostic dilemma. Autoimmune lymphoproliferative syndrome (ALPS) is a human genetic disorder of lymphocyte apoptosis resulting in an accumulation of lymphocytes and childhood onset chronic lymphadenopathy, splenomegaly, multilineage cytopenias, and an increased risk of B-cell lymphoma. In 1999, investigators at the National Institutes of Health (NIH) suggested criteria to establish the diagnosis of ALPS. Since then, with approximately 500 patients with ALPS studied worldwide, significant advances in our understanding of the disease have prompted the need for revisions to the existing diagnostic criteria and classification scheme. The rationale and recommendations outlined here stem from an international workshop held at NIH on September 21 and 22, 2009, attended by investigators from the United States, Europe, and Australia engaged in clinical and basic science research on ALPS and related disorders. It is hoped that harmonizing the diagnosis and classification of ALPS will foster collaborative research and better understanding of the pathogenesis of autoimmune cytopenias and B-cell lymphomas.

Indexed as

Autoimmune Lymphoproliferative SyndromeChildHumansUnited States

Identifiers

PMID20538792
PMCPMC2953894
OpenAlexW2047210789

What OpenQuestion holds

Textmetadata
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.