title: Paroxysmal nocturnal haemoglobinuria at Oslo University Hospital 2000-2010.
abstract: BACKGROUND
  Paroxysmal nocturnal haemoglobinuria (PNH) is a rare haematological disease characterised by chronic haemolysis, pancytopenia and venous thrombosis. The condition is attributable to a lack of control of complement attack on erythrocytes, thrombocytes and leukocytes, and can be diagnosed by means of flow cytometry. In this quality assurance study, we have reviewed information from the medical records of all patients tested for PNH using flow cytometry at our laboratory over a ten-year period.
  
  
  METHODS
  In the period 2000-2010 a total of 28 patients were tested for PNH using flow cytometry at the Department of Immunology and Transfusion Medicine, Oslo University Hospital. We have reviewed the results of these examinations retrospectively together with information from medical records and transfusion data for the patients concerned.
  
  
  RESULTS
  Flow cytometry identified 22 patients with PNH: four with classic disease and 18 with PNH secondary to another bone marrow disease. Five patients had atypical thrombosis. Seventeen patients received antithymocyte globulin or drug treatment; of these, six recovered from their bone marrow disease, while six died and five had a need for long-term transfusion. Five patients with life-threatening bone marrow disease underwent allogeneic stem cell transplantation, three of whom died. Six of 22 patients received eculizumab; the need for transfusion has been reduced or eliminated in three patients treated with eculizumab over a longer period.
  
  
  CONCLUSIONS
  Flow cytometry identified PNH in a majority of patients from whom we obtained samples. Most patients had a PNH clone secondary to bone marrow failure. Atypical thrombosis should be borne in mind as an indication for the test. Treatment with eculizumab is relevant for selected patients with PNH.
authors.affiliation: Avdeling for immunologi og transfusjonsmedisin Oslo universitetssykehus.
authors.forename: Lise Sofie H
authors.initials: LS
authors.lastname: Nissen-Meyer
authors.affiliation: Avdeling for blodsykdommer Oslo universitetssykehus.
authors.forename: Geir E
authors.initials: GE
authors.lastname: Tjønnfjord
authors.affiliation: Avdeling for immunologi og transfusjonsmedisin Oslo universitetssykehus.
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Golebiowska
authors.affiliation: Avdelning för Klinisk Immunologi och Transfusionsmedicin Labmedicin Skåne Lund, Sverige og Prophylix Pharma AS Forskningsparken Tromsø
authors.forename: Jens
authors.initials: J
authors.lastname: Kjeldsen-Kragh
authors.affiliation: Avdeling for immunologi og transfusjonsmedisin Oslo universitetssykehus.
authors.forename: Çiğdem Akalın
authors.initials: ÇA
authors.lastname: Akkök
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D001855:Bone Marrow Diseases / Q000150:complications / Q000188:drug therapy / Q000601:surgery; D018958:CD55 Antigens / Q000097:blood; D018957:CD59 Antigens / Q000097:blood; D005260:Female; D005434:Flow Cytometry; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008297:Male; D009664:Norway; D011785:Quality Assurance, Health Care; D012189:Retrospective Studies
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000961:Antilymphocyte Serum; D018958:CD55 Antigens; D018957:CD59 Antigens; C481642:eculizumab