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SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery

Overview of attention for article published in Blood, October 2018
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  • In the top 5% of all research outputs scored by Altmetric
  • High Attention Score compared to outputs of the same age (98th percentile)
  • High Attention Score compared to outputs of the same age and source (97th percentile)

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Title
SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery
Published in
Blood, October 2018
DOI 10.1182/blood-2018-03-840702
Pubmed ID
Authors

Elie Haddad, Brent R. Logan, Linda M. Griffith, Rebecca H. Buckley, Roberta E. Parrott, Susan E. Prockop, Trudy N. Small, Jessica Chaisson, Christopher C. Dvorak, Megan Murnane, Neena Kapoor, Hisham Abdel-Azim, Imelda C. Hanson, Caridad Martinez, Jack J. H. Bleesing, Sharat Chandra, Angela R. Smith, Matthew E. Cavanaugh, Soma Jyonouchi, Kathleen E. Sullivan, Lauri Burroughs, Suzanne Skoda-Smith, Ann E. Haight, Audrey G. Tumlin, Troy C. Quigg, Candace Taylor, Blachy J. Dávila Saldaña, Michael D. Keller, Christine M. Seroogy, Kenneth B. Desantes, Aleksandra Petrovic, Jennifer W. Leiding, David C. Shyr, Hélène Decaluwe, Pierre Teira, Alfred P. Gillio, Alan P. Knutsen, Theodore B. Moore, Morris Kletzel, John A. Craddock, Victor Aquino, Jeffrey H. Davis, Lolie C. Yu, Geoffrey D. E. Cuvelier, Jeffrey J. Bednarski, Frederick D. Goldman, Elizabeth M. Kang, Evan Shereck, Matthew H. Porteus, James A. Connelly, Thomas A. Fleisher, Harry L. Malech, William T. Shearer, Paul Szabolcs, Monica S. Thakar, Mark T. Vander Lugt, Jennifer Heimall, Ziyan Yin, Michael A. Pulsipher, Sung-Yun Pai, Donald B. Kohn, Jennifer M. Puck, Morton J. Cowan, Richard J. O'Reilly, Luigi D. Notarangelo

Abstract

The Primary Immune Deficiency Treatment Consortium performed a retrospective analysis of 662 patients with Severe Combined Immunodeficiency (SCID) who received an Hematopoietic cell transplantation (HCT) as first-line treatment between 1982 and 2012 in 33 North American institutions. Overall survival was higher after HCT from matched sibling donors (MSD). Among recipients of non-MSD HCT, multivariate analysis showed that SCID genotype strongly influenced survival and immune reconstitution. Overall survival was similar for patients with RAG, IL2RG or JAK3 defects, and was significantly better compared to patients with ADA or DCLRE1C mutations. Patients with RAG or DCLRE1C mutations had poorer immune reconstitution than other genotypes. Although survival did not correlate with the type of conditioning regimen, recipients of reduced intensity or myeloablative conditioning had a lower incidence of treatment failure and better T and B cell reconstitution, but higher risk of graft versus host disease compared with those receiving no conditioning or immunosuppression only. Infection-free status and younger age at HCT were each associated with improved survival. Typical SCID, leaky SCID and Omenn syndrome had similar outcomes. Landmark analysis identified CD4+ and CD4+CD45RA+ cell counts 6 and 12 months post-HCT as biomarkers predictive of overall survival and long-term T cell reconstitution. Our data emphasize the need for patient-tailored treatment strategies depending upon the underlying SCID genotype. The prognostic significance of CD4+ cell counts as early as 6 months after HCT emphasizes the importance of close follow-up of immune reconstitution to identify patients who may need additional intervention to prevent poor long-term outcome.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 51 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 51 100%

Demographic breakdown

Readers by professional status Count As %
Other 8 16%
Researcher 6 12%
Student > Master 6 12%
Student > Ph. D. Student 5 10%
Professor > Associate Professor 3 6%
Other 6 12%
Unknown 17 33%
Readers by discipline Count As %
Medicine and Dentistry 18 35%
Immunology and Microbiology 5 10%
Biochemistry, Genetics and Molecular Biology 3 6%
Nursing and Health Professions 2 4%
Chemical Engineering 1 2%
Other 6 12%
Unknown 16 31%

Attention Score in Context

This research output has an Altmetric Attention Score of 124. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 22 January 2019.
All research outputs
#140,510
of 14,511,573 outputs
Outputs from Blood
#77
of 18,188 outputs
Outputs of similar age
#5,340
of 273,579 outputs
Outputs of similar age from Blood
#6
of 225 outputs
Altmetric has tracked 14,511,573 research outputs across all sources so far. Compared to these this one has done particularly well and is in the 99th percentile: it's in the top 5% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 18,188 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 7.3. This one has done particularly well, scoring higher than 99% of its peers.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 273,579 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 98% of its contemporaries.
We're also able to compare this research output to 225 others from the same source and published within six weeks on either side of this one. This one has done particularly well, scoring higher than 97% of its contemporaries.