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Clinical Analysis of 70 Adult Patients with Paroxysmal Nocturnal Hemoglobinuria / 中国实验血液学杂志
Article de En | WPRIM | ID: wpr-357273
Bibliothèque responsable: WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinical characteristics of Chinese patients with paroxysmal nocturnal hemoglobinuria (PNH).</p><p><b>METHODS</b>The clinical data of 70 adult PNH cases in our hospital from January 2000 to December 2009 were analyzed retrospectively, and the clinical manifestation, laboratory examination, treatment, complications and prognostic factors influencing survival rate were assessed.</p><p><b>RESULTS</b>The nosopoietic median age of 70 cases(41 male cases and 29 female cases) was 37 (18-73) years old. The clinical manifestation included fatigue (87.1%), hemogolobinuria (44.3%), infection (22.9%), bleeding (37.1%), and abdominal pain (2.9%). FHb (free hemoglobin) in 56 patients (80%) was <50 mg/L. Hp (haptoglobin) in 54 patients (77.1%) was <0.5 g/L, and LDH in 49 patients (70.0%) was <220 U/L. The overall 10 year-survival rate after diagnosis was 72.2% estimated by Kaplan-Meier. The complications in this study were as follow: recurrent abdominal pain crisis (2.9%), infections (30.0%), thrombotic events (8.6%), evolution to MDS/AML (5.7%), calculus (11.4%) and death (17.1%). Both univariate and multivariate analyses identified risk factors affecting survival, including development of thrombotic events, progression to myelodysplastic syndrome or acute myelogenous leukemia (MDS/AML) and recurrent infections.</p><p><b>CONCLUSION</b>This larger number of cases for the first time allowed us to carry out a detailed analysis of prognostic factors for this rare disease. Evaluation of PNH prognostic factors may provide a basis to assess the current and future therapies of this disease.</p>
Sujet(s)
Texte intégral: 1 Indice: WPRIM Sujet Principal: Thrombose / Syndromes myélodysplasiques / Leucémie aigüe myéloïde / Analyse multifactorielle / Études rétrospectives / Facteurs de risque / Évolution de la maladie / Asiatiques / Hémoglobinurie paroxystique Type d'étude: Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limites du sujet: Adolescent / Adult / Aged / Female / Humans / Male langue: En Texte intégral: Journal of Experimental Hematology Année: 2015 Type: Article
Texte intégral: 1 Indice: WPRIM Sujet Principal: Thrombose / Syndromes myélodysplasiques / Leucémie aigüe myéloïde / Analyse multifactorielle / Études rétrospectives / Facteurs de risque / Évolution de la maladie / Asiatiques / Hémoglobinurie paroxystique Type d'étude: Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Limites du sujet: Adolescent / Adult / Aged / Female / Humans / Male langue: En Texte intégral: Journal of Experimental Hematology Année: 2015 Type: Article