血红蛋白病患者新冠病毒病的初步数据：一项多中心ICET-A研究
Preliminary Data on COVID-19 in Patients with Hemoglobinopathies: A Multicentre ICET-A Study.
作者信息
de Sanctis Vincenzo, Canatan Duran, Corrons Joan Lluis Vives, Karimi Mehran, Daar Shahina, Kattamis Christos, Soliman Ashraf T, Wali Yasser, Alkindi Salam, Huseynov Valeh, Nasibova Afag, Tiryaki Tarık Onur, Evim Melike Sezgin, Gunes Adalet Meral, Karakas Zeynep, Christou Soteroula, Campisi Saveria, Zarei Tahereh, Khater Doaa, Oymak Yesim, Kaleva Valeriya, Stoyanova Denka, Banchev Atanas, Galati Maria Concetta, Yassin Mohamed A, Kakar Shruti, Skafida Myrto, Kilinc Yurdanur, Alyaarubi Saif, Verdiyevas Narmin, Stoeva Iva, Raiola Giuseppe, Mariannis Demetris, Ruggiero Leopoldo, Di Maio Salvatore
机构信息
Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.
Antalya Genetic Diseases Diagnostic Center, Antalya, Turkey.
出版信息
Mediterr J Hematol Infect Dis. 2020 Jul 1;12(1):e2020046. doi: 10.4084/MJHID.2020.046. eCollection 2020.
OBJECTIVES
This study aims to investigate, retrospectively, the epidemiological and clinical characteristics, laboratory results, radiologic findings, and outcomes of COVID-19 in patients with transfusion-dependent β thalassemia major (TM), β-thalassemia intermedia (TI) and sickle cell disease (SCD).
DESIGN
A total of 17 Centers, from 10 countries, following 9,499 patients with hemoglobinopathies, participated in the survey.
MAIN OUTCOME DATA
Clinical, laboratory, and radiologic findings and outcomes of patients with COVID-19 were collected from medical records and summarized.
RESULTS
A total of 13 patients, 7 with TM, 3 with TI, and 3 with SCD, with confirmed COVID-19, were identified in 6 Centers from different countries. The overall mean age of patients was 33.7±12.3 years (range:13-66); 9/13 (69.2%) patients were females. Six patients had pneumonia, and 4 needed oxygen therapy. Increased C-reactive protein (6/10), high serum lactate dehydrogenase (LDH; 6/10), and erythrocyte sedimentation rate (ESR; 6/10) were the most common laboratory findings. 6/10 patients had an exacerbation of anemia (2 with SCD). In the majority of patients, the course of COVID-19 was moderate (6/10) and severe in 3/10 patients. A 30-year-old female with TM, developed a critical SARS-CoV-2 infection, followed by death in an Intensive Care Unit. In one Center (Oman), the majority of suspected cases were observed in patients with SCD between the age of 21 and 40 years. A rapid clinical improvement of tachypnea/dyspnea and oxygen saturation was observed, after red blood cell exchange transfusion, in a young girl with SCD and worsening of anemia (Hb level from 9.2 g/dl to 6.1g/dl).
CONCLUSIONS
The data presented in this survey permit an early assessment of the clinical characteristics of COVID 19 in different countries. 70% of symptomatic patients with COVID- 19 required hospitalization. The presence of associated co-morbidities can aggravate the severity of COVID- 19, leading to a poorer prognosis irrespective of age.
目的
本研究旨在回顾性调查依赖输血的重型β地中海贫血（TM）、中间型β地中海贫血（TI）和镰状细胞病（SCD）患者中新型冠状病毒肺炎（COVID-19）的流行病学和临床特征、实验室检查结果、影像学表现及预后。
设计
来自10个国家的17个中心对9499例血红蛋白病患者进行了随访，并参与了此次调查。
主要结局数据
从病历中收集并总结COVID-19患者的临床、实验室和影像学检查结果及预后。
结果
在来自不同国家的6个中心共确诊了13例COVID-19患者，其中7例为TM，3例为TI，3例为SCD。患者的总体平均年龄为33.7±12.3岁（范围：13 - 66岁）；9/13（69.2%）为女性。6例患者患有肺炎，4例需要吸氧治疗。最常见的实验室检查结果为C反应蛋白升高（6/10）、血清乳酸脱氢酶（LDH）升高（6/10）和红细胞沉降率（ESR）升高（6/10）。6/10的患者出现贫血加重（2例为SCD）。大多数患者的COVID-19病程为中度（6/10），3/10为重度。一名30岁的TM女性患者发生了严重的SARS-CoV-2感染，随后在重症监护病房死亡。在一个中心（阿曼），大多数疑似病例出现在21至40岁的SCD患者中。一名患有SCD且贫血加重（血红蛋白水平从9.2 g/dl降至6.1g/dl）的年轻女孩在进行红细胞置换输血后，呼吸急促/呼吸困难和血氧饱和度迅速得到临床改善。
结论
本次调查所呈现的数据有助于对不同国家COVID-19的临床特征进行早期评估。70%有症状的COVID-19患者需要住院治疗。合并症的存在会加重COVID-19的严重程度，导致无论年龄大小预后都较差。
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