![]() PNH testing had been performed by classical cytometry technique until 2010 and, thereafter, using high sensitivity (≥0.01%) FLAER-based assay according to 2010 International Clinical Cytometry Society (ICCS) PNH Consensus Guidelines and 2012 Practical PNH Guidelines. Bone marrow data were available for all patients classified as MDS (either hypoplastic or not), AA, leukemia, and myeloproliferative neoplasms (MPN). Blood counts were collected at the time of PNH clone testing, hence the positive impact of transfusions on baseline hemoglobin and platelets cannot be excluded in transfusion-dependent patients. Demographic and clinical phenotypes were retrospectively evaluated from August 2017 until January 2018.Ĭlinical history, blood counts (hemoglobin Hb, platelets Plt, absolute neutrophil counts ANC), hemolytic parameters, bone marrow features, disease-specific categories (WHO MDS categories), severity scores (Camitta criteria for AA, international prognostic scoring system IPSS and IPSS-revised for MDS), number and type of therapeutic interventions and their response rates, occurrence of complications (particularly thrombosis), disease progression/evolution, and death were collected. Overall, 3085 patients ( n = 3085) with a first time test for PNH at King’s College Hospital in London from March 1998 until October 2017 were included in the analysis. We correlated clone size with disease category (particularly AA and myelodysplastic syndromes, MDS), clinical and laboratory features, number, type and response to therapies, and with the occurrence of thrombosis, disease progression/evolution, and survival. The significance of subclinical, small PNH clones (50%) by flow cytometry with FLAER in an unselected population referred to a tertiary hematology center in UK. ![]() Since the last 10 years, a novel cytofluorimetric technique (fluorescent aerolysin (FLAER)-based assay) has been routinely used to detect PNH clones, with a sensitivity of ≥0.01% clone size. ![]() The rate of positivity varies greatly between 4 and 40% largely depending on the method and on the sensitivity. The presence of paroxysmal nocturnal hemoglobinuria (PNH) clones in bone marrow failure syndromes has been demonstrated by various investigators in heterogeneous series. Our data suggest systematic PNH testing in AA/MDS, as it might allow better prediction/prognostication and consequent clinical/laboratory follow-up timing. 12.7%, p = 0.01 in MDS), and overall survival, with a relative HR for mortality of 2.37 (95% CI 1.8–3.1 p < 0.0001) in PNH negative cases, both in univariate and multivariable analysis. 6.9% progression to MDS in AA, p = 0.01), leukemic evolution (6.8 vs. PNH positivity had a favorable impact on disease progression (0.6% vs. PNH clone, irrespective of size, was a good predictor of response to immunosuppressive therapy (IST) and to stem cell transplant (HSCT) (in MDS: 84% if PNH+ vs. PNH clones were more frequent and larger in AA vs. Given PNH association with bone marrow failures, we analyzed 869 myelodysplastic syndromes (MDS) and 531 aplastic anemia (AA) within the cohort. In this large single-centre study, we report high prevalence (25%) of, small (<10%) and very small (<1%), paroxysmal nocturnal hemoglobinuria (PNH) clones by high-sensitive cytometry among 3085 patients tested. ![]()
0 Comments
Leave a Reply. |
Details
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |