These results justify the need for PNH testing that can detect small PNH clones with at least 0.01% sensitivity in patients with AA/low-grade MDS. AA patients harboring small PNH clones show better responsiveness to immunosuppressive therapy, and 10–25% of these patients exhibit expansion of small PNH clones, which could progress to overt PNH. Small PNH clones are present in patients with aplastic anemia (AA) and low-grade myelodysplastic syndrome (MDS), with an incidence of 18.5% (AA)/1.1% (MDS) when a 1% cutoff is applied and 39.5% (AA)/1.8% (MDS) when a 0.01% cutoff is applied. Patients with classical PNH with overt (>1%) PNH clones diagnosed by flow cytometry (FCM) can show intravascular hemolysis however, symptom positivity is correlated with the proportion of cells lacking GPI-anchored proteins. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell disease caused by mutation of the PIG-A gene encoding enzymes involved in the biosynthesis of glycosylphosphatidylinositol (GPI)-anchored proteins on red blood cells (RBCs) and white blood cells (WBCs). Keywords: Flow cytometry, High sensitivity, Paroxysmal nocturnal hemoglobinuria, Performance, Clone HS-FCM can sensitively detect minor PNH clones and reduce false-positive C-FCM minor PNH clone cases in AA/low-grade MDS patients. In PNH patients, C-FCM detected a greater PNH clone size than HS-FCM (mean difference: 2.5%). C-FCM detected minor PNH clones in nine samples, but six of them were negative by HS-FCM. In AA/low-grade MDS patients, C-FCM showed >1% PNH clones in six samples, but HS-FCM showed >1% PNH clones in none of the samples. For red blood cells, C-FCM detected a greater PNH clone size than HS-FCM (mean difference: 1.5%). In PNH patients, C-FCM detected a smaller PNH clone size than HS-FCM (mean difference: 1.9?5.0%). Seven samples showed minor PNH clones by C-FCM, but HS-FCM showed negative results for all these samples. In AA/low-grade MDS patients, three samples showed >1% PNH clones with C-FCM but not with HS-FCM. For granulocytes, C-FCM detected a smaller PNH clone size than HS-FCM (mean difference: 0.7?1.7%). No healthy control samples had PNH clone size >0.01%. MethodsĬ-FCM and HS-FCM were performed simultaneously on 41 samples from healthy controls and 23 peripheral blood samples from 15 AA/low-grade MDS and eight PNH patients, using a Navios flow cytometer (Beckman Coulter, Miami, FL, USA). We compared its performance with conventional flow cytometry (C-FCM) for diagnosing overt PNH and detecting minor (0.1?1%) PNH clones in aplastic anemia (AA)/low-grade myelodysplastic syndrome (MDS) patients. High sensitivity flow cytometry (HS-FCM) was recently developed for diagnosing paroxysmal nocturnal hemoglobinuria (PNH).
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