Detection of paroxysmal nocturnal hemoglobinuria clones with flaer method for atypical located thrombosis, aplastic anemia, myelodysplastic syndrome patients

BACKGROUND: Paroxysmal Nocturnal Hemoglobinuria (PNH) is a clonal hematopoetic stem cell disease with intravascular hemolysis sympthoms, ineffectivity of bone marrow and thrombosis. In some patients PNH is single disease but it can be seen with aplastic anemia or myelodysplastic syndrome (MDS). Atypical located thromboembolies of PNH are the main causes of mortality and morbidity of disease. AIM: In this study, we aimed to detect and compare PNH clone with fluorescent aerolysin (FLAER) test for atypical located venous thrombosis, aplastic anemia, MDS, bicytopenia or pancytopenia patients with unknown etiology and healthy people. METHODS: We evaluated prospectively, 22 aplastic anemia, 20 MDS, 24 atypical located thrombosis, 7 bicytopenia or pancytopenia patients with unknown etiology and 20 healthy volunteers, totally 93 people in this study. By using FLAER method, we detected PNH clones of 93 people in this study. And we compared with each other. RESULTS: We detected that 6 ( 20.7% ) of 29 patients hypocellular bone marrow (aplastic anemia) and bicytopenia or pancytopenia patients with unknown etiology had PNH clones (p<0,05). Lactate Dehydrogenase (LDH) level of PNH clone positive patients is 589?99 U/L and LDH level of healthy vollunteers is 270?0.24 U/L (p=0.046). Mean Corpuscular Volume (MCV) of PNH clone positive patients is 96?4.55 fentoliter (fl), MCV of healthy vollunteers is 85.9?0.68 fl (p=0.021). Mean plattelet volume (MPV) of patients with thrombosis is 9.1?0.21 fl, MPV of healthy volunteers is 8,5?0.24fl (p=0.049). CONCLUSIONS: In the hypocellular bone marrow (aplastic anemia) and bicytopenia or pancytopenia patients with unknown etiology groups, detected PNH clones positivity is high. For the other groups there is no detection of PNH clones. Hypocellular bone marrow and bicytopenia or pancytopenia with unknown etiology patients must be evaluated for diagnosis of PNH. However, we need extra studies with high number of these all groups in this subject. Key words: Aplastic Anemia, Fluorescent Aerolysine, Myelodisplastic Syndrome, Paroxysmal Nocturnal Hemoglobinuria, Thrombosis