Résumé
Background : Paroxysmal nocturnal hemoglobinuria (PNH) results due to decrease or absence of glycosylphosphatidylinositol-anchored (GPI) molecules, such as CD55 and CD59, from the surface of the affected cells. PNH-phenotype has been described in various hematological disorders, mainly aplastic anemia and myelodysplastic syndromes; recently it has been reported in patients with lymphoproliferative syndromes and multiple myeloma (MM). Materials and Methods : We evaluated the presence of CD55 negative and/or CD59 negative red blood cell (RBC) populations in newly diagnosed treatment naive-54 chronic lymphocytic leukemia (CLL) and 29 MM patients by flow cytometry. Results : PNH-phenotype was not reported in any patient; however, RBC populations deficient in CD55 were detected in 16.66% (9/54) CLL and 6.89% (2/29) MM patients. Clinical presentation or the hematological parameters did not show any relationship with the presence of CD55 deficient RBC population. Conclusion : Our study showed absence of PNH-phenotype in patients with CLL and MM; however, isolated CD55 deficient RBC were identified in both CLL and MM. Larger prospective studies by other centers, including simultaneous analysis of granulocytes for the presence of PNH-phenotype, are needed to corroborate these findings and to work out the mechanisms and the significance of the existence of this phenotype in these patients.
Sujets)
Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Antigènes CD55/analyse , Érythrocytes/composition chimique , Femelle , Hémoglobinurie paroxystique/diagnostic , Humains , Leucémie chronique lymphocytaire à cellules B/complications , Mâle , Adulte d'âge moyen , Myélome multiple/complicationsRésumé
A case of a 63-year-old patient with motor neurone disease (amyotrophic lateral sclerosis) with central sleep apnoea syndrome is being reported. His sleep architecture was fragmented with a high apnea-hypopnea index of 65 per hour and maximum oxygen-desaturation of 78 percent. Total correction of sleep pattern with nasal non-invasive ventilation (BiPAP-ST) was demonstrated.
Sujets)
Sclérose latérale amyotrophique/complications , Humains , Mâle , Adulte d'âge moyen , Polysomnographie , Ventilation à pression positive , Apnée centrale du sommeil/étiologieRésumé
Two patients with post-ventilatory tracheal stenosis were treated by repeated dilatation with oesophageal dilators under general anaesthesia, and direct vision of the fiberoptic bronchoscope. The results of dilatations were remarkable and the patients continue to be asymptomatic after a follow up of more than two years.