Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
5
|
| pubmed:dateCreated |
1994-9-28
|
| pubmed:abstractText |
We analyzed data from 388 subjects with Fanconi anemia reported to the International Fanconi Anemia Registry (IFAR). Of those, 332 developed hematologic abnormalities at a median age of 7 years (range, birth to 31 years). Actuarial risk of developing hematopoietic abnormalities was 98% (95% confidence interval, 93% to 99%) by 40 years of age. Common hematologic abnormalities were thrombocytopenia and pancytopenia. These were often associated with decreased bone marrow (BM) cellularity (75% of cases studied). Clonal cytogenetic abnormalities developed in 23 of 68 persons with BM failure who had adequate studies. Actuarial risk of clonal cytogenetic abnormalities during BM failure was 67% (47% to 87%) by 30 years of age. Fifty-nine subjects developed myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML). Actuarial risk of MDS or AML was 52% (37% to 67%) by 40 years of age. Risk was higher in persons with than in those without a prior clonal cytogenetic abnormality (3% [0% to 9%] v 35% [0% to 79%]; P = .006). One hundred twenty persons died of hematologic causes including BM failure, MDS or AML and treatment related complications. Actuarial risk of death from hematologic causes was 81% (67% to 90%) by 40 years of age.
|
| pubmed:grant | |
| pubmed:commentsCorrections | |
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
AIM
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Sep
|
| pubmed:issn |
0006-4971
|
| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
1
|
| pubmed:volume |
84
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
1650-5
|
| pubmed:dateRevised |
2007-11-14
|
| pubmed:meshHeading |
pubmed-meshheading:8068955-Adolescent,
pubmed-meshheading:8068955-Adult,
pubmed-meshheading:8068955-Age Factors,
pubmed-meshheading:8068955-Bone Marrow,
pubmed-meshheading:8068955-Child,
pubmed-meshheading:8068955-Child, Preschool,
pubmed-meshheading:8068955-Cytogenetics,
pubmed-meshheading:8068955-Fanconi Anemia,
pubmed-meshheading:8068955-Female,
pubmed-meshheading:8068955-Hematologic Diseases,
pubmed-meshheading:8068955-Humans,
pubmed-meshheading:8068955-Infant,
pubmed-meshheading:8068955-Infant, Newborn,
pubmed-meshheading:8068955-International Agencies,
pubmed-meshheading:8068955-Leukemia,
pubmed-meshheading:8068955-Male,
pubmed-meshheading:8068955-Myelodysplastic Syndromes,
pubmed-meshheading:8068955-Pancytopenia,
pubmed-meshheading:8068955-Registries,
pubmed-meshheading:8068955-Risk Factors,
pubmed-meshheading:8068955-Thrombocytopenia
|
| pubmed:year |
1994
|
| pubmed:articleTitle |
Hematologic abnormalities in Fanconi anemia: an International Fanconi Anemia Registry study.
|
| pubmed:affiliation |
Laboratory for Investigative Dermatology, Rockefeller University, New York, NY.
|
| pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
|