Analysis and summary of clinical characteristics of 289 patients with paroxysmal nocturnal hemoglobinuria in Zhejiang Province
10.3760/cma.j.cn121090-20240127-00041
- VernacularTitle:浙江省289例阵发性睡眠性血红蛋白尿症患者的临床特征
- Author:
Gaixiang XU
1
;
Weimei JIN
;
Baodong YE
;
Songfu JIANG
;
Chao HU
;
Xin HUANG
;
Bingshou XIE
;
Huifang JIANG
;
Lili CHEN
;
Rongxin YAO
;
Ying LU
;
Linjie LI
;
Jin ZHANG
;
Guifang OUYANG
;
Yongwei HONG
;
Hongwei KONG
;
Zhejun QIU
;
Wenji LUO
;
Binbin CHU
;
Huiqi ZHANG
;
Hui ZENG
;
Xiujie ZHOU
;
Pengfei SHI
;
Ying XU
;
Jie JIN
;
Hongyan TONG
Author Information
1. 浙江大学医学院附属第一医院，杭州 310003
- Keywords:
Paroxysmal nocturnal hemoglobinuria;
Lactic dehydrogenase;
Thrombus;
PNH clone
- From:
Chinese Journal of Hematology
2024;45(6):549-555
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.
