title: Paroxysmal nocturnal haemoglobinuria. Experience over a 10 years period.
abstract: BACKGROUND
  Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic, clonal and acquired disorder of the hematopoietic stem cell with a deficiency of all glycophosphatidyl-inositol (GPI) linked proteins. The aim of this retrospective study was to analyse haematological and biochemical data from 152 patients referred to our laboratory for diagnosis of PNH by flow cytometry (FC).
  
  
  METHODS
  Patients and healthy donor (152 and 99 respectively) were studied. Ham, sucrose, lactate dehydrogenase (LDH), Iron, haptoglobin (Hp), blood cell morphology and Kaplow cytochemical stain for leukocyte alkaline phosphatase (LAP) were carried out. GPI-proteins anti-CD55 and CD59 in erythrocytes and the former, plus anti CD16b and CD66b on neutrophils were evaluated by FC.
  
  
  RESULTS
  Anemia and/or leukopenia and/or thrombocytopenia, increased reticulocyte count and LDH were observed in patients with PNH clone. Some of them had dacriocytes, schistocytes. LAP was low. On average, we detected 50% CD59 (-) erythrocytes and 29, 83, 78% CD55/59 (-), CD16b (-), CD66b (-) neutrophils, respectively.
  
  
  CONCLUSIONS
  Paroxysmal nocturnal hemoglobinuria clone was detected in 20/152 patients. Negative population's percentages were high in patients with classic PNH, Hematimetry, LAP and adequate use of CF contribute to PNH clone detection in the laboratory.
authors.affiliation: Apoyo Médico, Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina.
authors.forename: K
authors.initials: K
authors.lastname: Canalejo
authors.forename: N
authors.initials: N
authors.lastname: Riera Cervantes
authors.forename: M
authors.initials: M
authors.lastname: Felippo
authors.forename: C
authors.initials: C
authors.lastname: Sarandría
authors.forename: M
authors.initials: M
authors.lastname: Aixalá
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000378:metabolism; D001772:Blood Cell Count; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D060965:Clonal Evolution; D004909:Erythrocyte Indices; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D005500:Follow-Up Studies; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood* / Q000175:diagnosis*; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D015703:Antigens, CD
keywords: PNH; hemolytic anemia; immunophenotypic analysis