The comparison of fluorescent aerolysin method with conventional diagnosis techniques in high risk paroxysmal nocturnal hemoglobinuria patients

Background and Aims: Paroxysmal nocturnal hemoglobinuria which is a clonal disorder of hematopoietic stem cells manifests itself with the classic signs of chronic intravascular hemolysis, bone marrow failure, and thrombosis. Some of the mutant proteins in the cell membrane that connects with the help of the glycophosphatidylinositol anchor product of hematopoietic stem cells are deficient. Fluorescent aerolysin has an excellent sensitivity. It determines Paroxysmal nocturnal hemoglobinuria clones with the rate of 0.01%.Methods: We have enrolled, 24 male (60%) and 16 female (40%), a total of 40 patients in our study. According to the initial diagnosis of patients, 4 patients (10%) have Paroxysmal nocturnal hemoglobinuria, 11 patients have (28%) aplastic anemia, 5 patients have (12%) hemolytic anemia, 12 patients have (30%) myelodysplastic syndrom and 8 patients (20%) have pancytopenia.Results: Patients, who had positive Fluorescent aerolysin test in the first diagnosis had Paroxysmal nocturnal hemoglobinuria 4 (100%), while 6 patients (55%) initial diagnosis of aplastic anemia, 1 patient (20%) patients with initial diagnosis of hemolytic anemia, and 1 (12%) of the initial diagnosis pancytopenia observed. All of the 12 patients with positive Fluorescent aerolysin results had CD 55 and CD-59 deficiency, whereas in 11 of 12 patients were Ham positive. CD 55 and CD 59 has been regarded as the gold standard for the diagnosis of Paroxysmal nocturnal hemoglobinuria, the sensitivity and specificity of Fluorescent aerolysin test was 100% for both criteria.Conclusion: Fluorescent aerolysin test is the most reliable sources among of the clinician in the diagnosis Paroxysmal nocturnal hemoglobinuria of among specific diagnostic methods.Key words: Fluorescent aerolysin, Paroxysmal nocturnal hemoglobinuria, Flow cytometry, Anemia, CD55 and CD59