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Small PNH clones detected by fluorescent aerolysin predict a faster response to immunosuppressive therapy in patients with severe aplastic anaemia.
- Source :
-
Hematology . Dec2020, Vol. 25 Issue 1, p348-355. 8p. - Publication Year :
- 2020
-
Abstract
- Objectives: To clear the obscure conclusion on the prediction value of paroxysmal nocturnal haemoglobinuria (PNH) clones in severe aplastic anaemia (SAA) patients treated with immunosuppressive therapy (IST). Methods: We retrospectively analyzed 219 consecutive SAA patients treated with IST from October 2008 to October 2015 and evaluated the haematological responses to IST. Results: The presence of a PNH clone was detected in 55 (25.1%) patients prior to IST [37/88 by flow cytometry (FCM) and 18/131 by fluorescent aerolysin (FLAER)] and 27 disappeared after IST (23/37 in initial FCM group, 4/18 in initial FLAER group, p = 0.005). In patients without an initial clone, 12 (30.0%) cases in FCM and 17 (19.5%) in FLAER groups presented a PNH clone at least once after IST (p < 0.001). In patients with a pre-treatment PNH clone detected by FCM, the 3-, 6- and 12-month response rates were higher than patients without (p = 0.006; 0.002 and 0.002, respectively). And in FLAER group, the 3-month response rate was significantly higher in those with a prior clone (p = 0.017), however, the 6- and 12-month response rates showed no differences (p = 0.105, p = 0.144, respectively). By multivariate analysis, a shorter interval between diagnosis and treatment is associated with a better response and survival. Conclusions: A more reliable FLAER method allows us to draw a conclusion that PNH clone predicts a faster response but not a higher response rate to IST. Once a diagnosis is confirmed, the IST should be initiated as soon as possible. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 10245332
- Volume :
- 25
- Issue :
- 1
- Database :
- Academic Search Index
- Journal :
- Hematology
- Publication Type :
- Academic Journal
- Accession number :
- 147735897
- Full Text :
- https://doi.org/10.1080/16078454.2020.1821495