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- W2972563314 abstract "Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is a rare life-threatening condition due to an acquired somatic mutation of the PIGA gene, leading to nonmalignant clonal expansion of hematopoietic stem cells, which are deficient in glycosyl phosphatidylinositol-anchored proteins (GPI-APs). Fluorescein-labeled proaerolysin (FLAER) and flow cytometry are key tools in the diagnosis of PNH. While clonal detection of PNH in both tests has a sensitive diagnostic threshold of 0.01% in erythrocytes and 0.05% to 1% in leukocytes, one must be cautious in ruling out the possibilities of myelodysplastic syndrome (MDS) or aplastic anemia. We propose guidelines in the differential diagnosis and evaluation of PNH from these and other hematologic disorders that can arise from GPI-AP deficient cells. These guidelines are based on a meta-analysis of five research literature sources, including four case studies. We also compare and contrast our limits of quantification of the in-house PNH assay at University of Kentucky Healthcare with those of an interlaboratory validation of 11 institutions within the United Kingdom. Our report advocates for thorough evaluation of multiple laboratory and clinical variables affecting sensitivity and accuracy of flow cytometry and FLAER in PNH. Furthermore, we recommend lowering of the in-house limit of quantification from the current 1% to 0.01%. This allows for the critical consideration of conditions such as MDS and aplastic anemia and their disease courses, all of which can present with PNH clones as low as 0.01% on flow cytometry and FLAER." @default.
- W2972563314 created "2019-09-19" @default.
- W2972563314 creator A5045958741 @default.
- W2972563314 date "2019-09-11" @default.
- W2972563314 modified "2023-09-27" @default.
- W2972563314 title "Paroxysmal Nocturnal Hemoglobinuria: Grasping the Flow of Diagnostic Dilemmas" @default.
- W2972563314 doi "https://doi.org/10.1093/ajcp/aqz117.008" @default.
- W2972563314 hasPublicationYear "2019" @default.
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