Detection of paroxysmal nocturnal hemoglobinuria (PNH) in bone marrow aspirates☆
Autor: | Irina Maric, Katherine R. Calvo, Alina Dulau-Florea, Raul C. Braylan |
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Rok vydání: | 2019 |
Předmět: |
biology
medicine.diagnostic_test Chemistry Cell Hemoglobinuria Paroxysmal Aerolysin Hematology CD59 Flow Cytometry medicine.disease Molecular biology Flow cytometry Haematopoiesis medicine.anatomical_structure Bone Marrow hemic and lymphatic diseases Paroxysmal nocturnal hemoglobinuria medicine biology.protein Humans Bone marrow Antibody |
Zdroj: | Seminars in Hematology. 56:65-68 |
ISSN: | 0037-1963 |
Popis: | Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired condition in which, due to a mutation of the phosphatidylinositol glycan class A gene, hematopoietic cells lack proteins that are normally anchored to the cell surface by glycosylphosphatidylinositol (GPI). Thus, PNH cells show poor expression of surface proteins, such as CD55 and CD59, and dim or absent binding of fluorescently labeled modified aerolysin (FLAER). In clinical diagnostic laboratories, the detection and quantitation of PNH is currently performed by flow cytometry (FC) analysis of peripheral blood (PB) samples. Although PB remains the preferred source of cells for PNH detection, we and other authors have shown that a careful FC analysis of bone marrow (BM) aspirates can provide results for PNH detection and quantitation equivalent to those obtained with PB. Here, we review studies delineating the expression of GPI-anchored proteins and FLAER binding in normal BM cells, and summarize published findings demonstrating the feasibility of identifying and quantitating PNH cells in BM using FLAER as well as antibodies against GPI-anchored proteins. Detection of PNH cells in BM should be useful in patients with unsuspected PNH or with severe cytopenia in whom PB FC analysis may be challenging. |
Databáze: | OpenAIRE |
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