| Title |
Hematopoietic Stem Cell Transplantation for X-Linked Thrombocytopenia With Mutations in the WAS gene
|
|---|---|
| Published in |
Journal of Clinical Immunology, November 2014
|
| DOI | 10.1007/s10875-014-0105-5 |
| Pubmed ID | |
| Authors |
Koichi Oshima, Kohsuke Imai, Michael H. Albert, Tanja C. Bittner, Gabriele Strauss, Alexandra H. Filipovich, Tomohiro Morio, Neena Kapoor, Jignesh Dalal, Kirk R. Schultz, James T. Casper, Luigi D. Notarangelo, Hans D. Ochs, Shigeaki Nonoyama |
| Abstract |
X-linked thrombocytopenia (XLT) is a mild form of the Wiskott-Aldrich syndrome (WAS) caused by mutations in the WAS gene. A recent retrospective study of the clinical outcome and molecular basis of a large cohort of XLT patients demonstrated that although overall survival is excellent, event free survival is severely affected with conservative treatment. To answer the question whether hematopoietic stem cell transplantation (HSCT) offers a viable alternative therapeutic option in XLT, we retrospectively investigated the outcome of HSCT in a cohort of 24 XLT patients who received HSCT between 1990 and 2011 at 14 transplant centers in the United States, Italy, Germany, Canada, and Japan. The engraftment rate was 100 % and the overall survival rate was 83.3 %. Of the four non-survivors, 2 underwent splenectomy prior to HSCT and died of sepsis, and two of aspergillus infections associated with severe GVHD. In all but one patient, pretransplant complications were resolved by HSCT. Our data indicate that HSCT following myeloablative conditioning is curative and associated with acceptable risks as a treatment option for XLT. |
X Demographics
The data shown below were collected from the profiles of 3 X users who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 3 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Science communicators (journalists, bloggers, editors) | 2 | 67% |
| Members of the public | 1 | 33% |
Mendeley readers
The data shown below were compiled from readership statistics for 33 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 1 | 3% |
| Unknown | 32 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 4 | 12% |
| Student > Postgraduate | 4 | 12% |
| Other | 3 | 9% |
| Student > Master | 3 | 9% |
| Student > Doctoral Student | 2 | 6% |
| Other | 4 | 12% |
| Unknown | 13 | 39% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 8 | 24% |
| Biochemistry, Genetics and Molecular Biology | 5 | 15% |
| Agricultural and Biological Sciences | 3 | 9% |
| Pharmacology, Toxicology and Pharmaceutical Science | 1 | 3% |
| Engineering | 1 | 3% |
| Other | 0 | 0% |
| Unknown | 15 | 45% |
Attention Score in Context
This research output has an Altmetric Attention Score of 3. 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 23 November 2014.
All research outputs
#13,341,515
of 22,770,070 outputs
Outputs from Journal of Clinical Immunology
#859
of 1,556 outputs
Outputs of similar age
#122,937
of 258,738 outputs
Outputs of similar age from Journal of Clinical Immunology
#6
of 18 outputs
Altmetric has tracked 22,770,070 research outputs across all sources so far. This one is in the 41st percentile – i.e., 41% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,556 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.4. This one is in the 44th percentile – i.e., 44% of its peers scored the same or lower than it.
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 258,738 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 52% of its contemporaries.
We're also able to compare this research output to 18 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 66% of its contemporaries.