The importance of flow cytometry in the diagnosis and monitoring of paroxysmal nocturnal hemoglobinuria

ABSTRACT Introduction: The paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disease of the hematopoietic stem cells, and it clinically characterized by chronic intravascular hemolysis, bone marrow failure hypercoagulability leading to thrombosis. It a rare disorder cells that occurs due somatic mutation in gene phosphatidylinositol glycan class A (PIG-A). Objective: Here we reviewed importance screening monitoring individuals with high risk developing PNH, since early diagnosis essential for better prognostic treatment choice patient. Method: review was carried out great focus on pathophysiology mainly use flow cytometry technique detect disease. Results: This codifies enzyme formation glycosylphosphatidylinositol (GPI), which acts as molecular anchor many membrane proteins. alteration GPI synthesis promotes partial or complete loss proteins needs this bind cell surface. Among these are CD55 CD59, control activation complement cascade. Conclusion: immunophenotyping exam considered reference test PNH diagnosis, highly sensitive specific, presenting advantages quantitative identification small populations phenotype capacity distinguish total deficiency GPI-anchored