Source:http://linkedlifedata.com/resource/pubmed/id/18535202
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
8
|
| pubmed:dateCreated |
2008-10-8
|
| pubmed:abstractText |
The natural history of paroxysmal nocturnal hemoglobinuria (PNH) clinical subcategories (classic PNH and aplastic anemia [AA]/PNH syndrome) is still unknown. We retrospectively studied 460 PNH patients diagnosed in 58 French hematologic centers from 1950 to 2005. The median (SE) follow-up time was 6.8 (0.5) years. The median survival time (SE) was 22 (2.5) years. We identified 113 patients with classic PNH, 224 patients with AA-PNH syndrome, and 93 (22%) intermediate patients who did not fit within these 2 categories. At presentation, classic PNH patients were older, with more frequent abdominal pain and displayed higher levels of GPI-AP-deficient granulocytes. A time-dependent improved survival was observed. In classic PNH, diagnoses before 1986 (hazard ratio [HR]: 3.6; P = .01) and increasing age (P < .001) were associated with worse survival prognoses, whereas use of androgens within the first year after diagnosis was protective (HR, 0.17; P = .01). Transfusions before 1996 (HR, 2.7; P = .007) led to lower survival rates in patients with AA-PNH syndrome, whereas immunosuppressive treatment was associated with better outcomes (HR, 0.33; P = .03). Evolution to thrombosis affected survival in both subcategories (classic PNH: HR, 7.8 [P < .001]; AA-PNH syndrome: HR, 33.0 [P < .001]). Evolution to bicytopenia or pancytopenia for classic PNH (HR, 7.3, P < .001) and malignancies for AA-PNH syndrome (HR, 48.8; P < .001) were associated with worse outcomes. Although clinical presentation and prognosis factors are different, classic PNH and AA-PNH syndrome present roughly similar outcomes, affected mainly by complications.
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| pubmed:language |
eng
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| pubmed:journal | |
| pubmed:citationSubset |
AIM
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| pubmed:chemical | |
| pubmed:status |
MEDLINE
|
| pubmed:month |
Oct
|
| pubmed:issn |
1528-0020
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| pubmed:author |
pubmed-author:CahnJean YvesJY,
pubmed-author:EtienneGabrielG,
pubmed-author:French Association of Young Hematologists,
pubmed-author:French Society of Hematology,
pubmed-author:MaryJean YvesJY,
pubmed-author:MaurySebastienS,
pubmed-author:MohtyMohamadM,
pubmed-author:RothSophieS,
pubmed-author:SalanoubatCéliaC,
pubmed-author:SociéGerardG,
pubmed-author:TerriouLouisL,
pubmed-author:de GuibertSophieS,
pubmed-author:de LatourRégis PeffaultRP
|
| pubmed:issnType |
Electronic
|
| pubmed:day |
15
|
| pubmed:volume |
112
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
3099-106
|
| pubmed:meshHeading |
pubmed-meshheading:18535202-Adult,
pubmed-meshheading:18535202-Female,
pubmed-meshheading:18535202-Hematology,
pubmed-meshheading:18535202-Hemoglobinuria, Paroxysmal,
pubmed-meshheading:18535202-Humans,
pubmed-meshheading:18535202-Immunosuppressive Agents,
pubmed-meshheading:18535202-Male,
pubmed-meshheading:18535202-Middle Aged,
pubmed-meshheading:18535202-Multivariate Analysis,
pubmed-meshheading:18535202-Prognosis,
pubmed-meshheading:18535202-Proportional Hazards Models,
pubmed-meshheading:18535202-Risk,
pubmed-meshheading:18535202-Thrombosis,
pubmed-meshheading:18535202-Time Factors,
pubmed-meshheading:18535202-Treatment Outcome
|
| pubmed:year |
2008
|
| pubmed:articleTitle |
Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories.
|
| pubmed:affiliation |
Service d'Hématologie-Greffe, Université Paris 7, France.
|
| pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|