AI Summary & Key Takeaways
AI InsightThere is a need to elucidate the relationship between hematological malignancies and PNH with the help of more advanced molecular techniques.
📌 3 Key Takeaways
Abstract
Objective: To investigate the frequency of paroxysmal nocturnal hemoglobinuria (PNH), an acquired clonal hematopoietic stem cell disease resulting in complement-mediated hemolysis, in patients with lymphoma by flow cytometry.Methods: Fifty patients with lymphoma who were admitted to the hematology clinic, newly diagnosed and not yet treated were included in this study conducted in 2014. The presence of PNH clones was checked by FLAER flow cytometry method in peripheral blood samples. FLAER is a non-hemolytic fluorescently labeled inactive toxin aerolysin method that can detect up to 0.5% of PNH cells instead of bacterial toxin aerolysin, which binds to RBCs via the GPI anchor and initiates hemolysis for PNH screening or PNH clone detection. With this technique, PNH clones in all hematopoietic cell lines can be detected in an assay.Results: PNH clone was observed over 10% in two patients, one male and the other female. However, no hemolysis was found in patients with PNH clones. The lymphoma subtypes of the patients with positive PNH clone were B-cell small-cell lymphocytic lymphoma in the male patient and primary splenic lymphoma in the female patient.Conclusion: PNH or PNH-like disorders accompanying hematological malignancies, especially lymphomas, are not very common in the literature. There is a need to elucidate the relationship between hematological malignancies and PNH with the help of more advanced molecular techniques.
Özet
Objective: To investigate the frequency of paroxysmal nocturnal hemoglobinuria (PNH), an acquired clonal hematopoietic stem cell disease resulting in complement-mediated hemolysis, in patients with lymphoma by flow cytometry.Methods: Fifty patients with lymphoma who were admitted to the hematology clinic, newly diagnosed and not yet treated were included in this study conducted in 2014. The presence of PNH clones was checked by FLAER flow cytometry method in peripheral blood samples. FLAER is a non-hemolytic fluorescently labeled inactive toxin aerolysin method that can detect up to 0.5% of PNH cells instead of bacterial toxin aerolysin, which binds to RBCs via the GPI anchor and initiates hemolysis for PNH screening or PNH clone detection. With this technique, PNH clones in all hematopoietic cell lines can be detected in an assay.Results: PNH clone was observed over 10% in two patients, one male and the other female. However, no hemolysis was found in patients with PNH clones. The lymphoma subtypes of the patients with positive PNH clone were B-cell small-cell lymphocytic lymphoma in the male patient and primary splenic lymphoma in the female patient.Conclusion: PNH or PNH-like disorders accompanying hematological malignancies, especially lymphomas, are not very common in the literature. There is a need to elucidate the relationship between hematological malignancies and PNH with the help of more advanced molecular techniques.