Prognostic value of pre-treatment PNH clone among the patients with aplastic anemia: a meta-analysis
J P Li, Xi Li, Lingxiao Cai, Xianghong Peng, Mengzhu Yao, Shuyan Li, Guoxiang Zhang
Abstract
Background Paroxysmal nocturnal hemoglobinuria (PNH) clone can be detected in some patients with aplastic anemia (AA) before treatment. But the prognostic value of the presence of pre-treatment PNH clone for intensive immunosuppressive therapy (IIST) is controversial and no consensus on whether the occurrence of PNH/AA-PNH syndrome is related to pre-treatment PNH clone.Objective This study aims to summarize the prognostic value of the presence of pre-treatment PNH clone treated with IIST among the AA patients and to elucidate its relationship with the development of PNH / AA-PNH syndrome.Methods All published studies on the prognostic value of pre-treatment PNH clone among AA patients were retrieved. Pooled odds ratio (OR) was calculated to compare the rates, along with 95% confidence intervals (CI) and p value to assess whether the results were statistically significant.Results The meta-analysis consisted of 15 studies with a combined total of 1349 patients in the cohort. Pre-treatment PNH clone had a positive effect on AA patients 6-month (pooled OR = 1.49,95% Cl: 1.06-2.08, P = 0.020), 12-month (pooled OR = 3.10,95% Cl: 1.89-5.10, P = 0.000), and overall hematological response rate (pooled OR = 1.69,95% Cl: 1.07-2.68, P = 0.024) after IIST. Patients with pre-treatment PNH clone are more likely to develop PNH/AA-PNH syndrome after IIST(pooled OR = 2.78,95%Cl:1.21-6.39, P = 0.016).Conclusion Patients with positive pre-treatment PNH clone had better hematological responses to IIST than negative. And, those patients are more likely to develop PNH/AA-PNH syndrome after IIST.