Clinical observation of low-dose combination chemotherapy in refractory/recurrent paroxysmal nocturnal hemoglobinuria patients: A single-center retrospective analysis.
Clinical observation of low-dose combination chemotherapy in refractory/recurrent paroxysmal nocturnal hemoglobinuria patients: A single-center retrospective analysis.
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DOI：
10.1002/jcla.24239
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发表时间：
2022-03
影响因子：
2.7
通讯作者：
Fu R
中科院分区：
文献类型：
--
作者：
Li L;Liu H;Wang H;Liu Z;Chen Y;Liu C;Zhao X;Li L;Wang H;Shao Z;Fu R
我们回顾性分析了20例难治性/复发性PNH患者的临床特点和治疗策略，包括化疗的临床疗效、安全性和生存率。分析我院20例难治性/复发性或糖皮质激素依赖的经典PNH患者的临床资料，包括临床表现、实验室检查、治疗效果及生存期。17例患者化疗后贫血症状明显改善，14例患者实现输血独立，3例患者Hb恢复正常。6例患者仍需输血，但输血间隔明显延长。溶血指标LDH、TBIL、RET的百分比均明显低于化疗前。与化疗前相比，肾上腺糖皮质激素的剂量减少了一半以上。化疗可减少PNH克隆，促进正常造血，控制溶血发作。这是一种很有前途和广泛应用的治疗方法。我们回顾性分析了20例难治性/复发性PNH患者的临床特点和治疗策略，包括化疗的临床疗效、安全性和生存率。分析我院20例难治性/复发性或糖皮质激素依赖的经典PNH患者的临床资料，包括临床表现、实验室检查、治疗效果及生存期。17例患者化疗后贫血症状明显改善，14例患者实现输血独立，3例患者Hb恢复正常。6例患者仍需输血，但输血间隔明显延长。溶血指标LDH、TBIL、RET的百分比均明显低于化疗前。与化疗前相比，肾上腺糖皮质激素的剂量减少了一半以上。化疗可减少PNH克隆，促进正常造血，控制溶血发作。这是一种很有前途和广泛应用的治疗方法。
We performed a retrospective analysis to investigate the clinical characteristics and therapeutic strategies of 20 refractory/recurrent PNH patients, including the clinical efficacy of chemotherapy treatment, safety, and survival. The clinical data of 20 classic PNH patients who were refractory/recurrent or had glucocorticoid dependence in our hospital were analyzed, including clinical manifestations, laboratory examinations, treatment efficacy, and survival. Seventeen patients had a marked improvement in anemia after chemotherapy, 14 patients acquired blood transfusion independence, and the Hb of 3 patients increased to normal levels. Although 6 patients still needed blood transfusion, the transfusion interval was significantly prolonged. The percentages of LDH, TBIL, and RET, which are indicators of hemolysis, were significantly lower than those before chemotherapy. The dosage of adrenal glucocorticoids was reduced by more than half compared with that before chemotherapy. Chemotherapy can reduce PNH clones, promote normal hematopoiesis, and control hemolytic attack. It is a promising and widely used therapeutic method. We performed a retrospective analysis to investigate the clinical characteristics and therapeutic strategies of 20 refractory/recurrent PNH patients, including the clinical efficacy of chemotherapy treatment, safety, and survival. The clinical data of 20 classic PNH patients that were refractory/recurrent or had glucocorticoid dependence in our hospital were analyzed, including clinical manifestations, laboratory examinations, treatment efficacy, and survival. Seventeen patients had a marked improvement in anemia after chemotherapy, 14 patients acquired blood transfusion independence, and the Hb of 3 patients increased to normal levels. Although 6 patients still needed blood transfusion, the transfusion interval was significantly prolonged. The percentages of LDH, TBIL, and RET, which are indicators of hemolysis, were significantly lower than those before chemotherapy. The dosage of adrenal glucocorticoids was reduced by more than half compared with that before chemotherapy. Chemotherapy can reduce PNH clones, promote normal hematopoiesis, and control hemolytic attack. It is a promising and widely used therapeutic method.
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DOI：
10.1038/nrdp.2017.28
发表时间：
2017-05-18
期刊：
Nature reviews. Disease primers
影响因子：
--
作者：
Hill A;DeZern AE;Kinoshita T;Brodsky RA
通讯作者：
Brodsky RA
影响因子：
2.6
作者：
Tian, Hong;Liu, Liming;Wu, Depei
通讯作者：
Wu, Depei
影响因子：
3.6
作者：
de Fontbrune, Flore Sicre;de latour, Regis Peffault
通讯作者：
de latour, Regis Peffault
影响因子：
4.4
作者：
Schatz-Jakobsen, Janus Asbjorn;Zhang, Yuchun;Andersen, Gregers Rom
通讯作者：
Andersen, Gregers Rom
影响因子：
2.8
作者：
Alfinito, Fiorella;Ruggiero, Giuseppina;Terrazzano, Giuseppe
通讯作者：
Terrazzano, Giuseppe