| Paroxysmal nocturnal hemoglobinuria: natural history of disease subcategories. | |
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MedLine Citation:
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PMID: 18535202 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Régis Peffault de Latour; Jean Yves Mary; Célia Salanoubat; Louis Terriou; Gabriel Etienne; Mohamad Mohty; Sophie Roth; Sophie de Guibert; Sebastien Maury; Jean Yves Cahn; Gerard Socié; ; |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2008-06-05 |
Journal Detail:
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Title: Blood Volume: 112 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2008 Oct |
Date Detail:
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Created Date: 2008-10-08 Completed Date: 2008-10-16 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 3099-106 Citation Subset: AIM; IM |
Affiliation:
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Service d'Hématologie-Greffe, Université Paris 7, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Female Hematology / methods Hemoglobinuria, Paroxysmal / classification*, complications, diagnosis* Humans Immunosuppressive Agents / therapeutic use Male Middle Aged Multivariate Analysis Prognosis Proportional Hazards Models Risk Thrombosis / genetics Time Factors Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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0/Immunosuppressive Agents |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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