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马来西亚成人多中心研究中初诊免疫性血小板减少症的临床流行病学、治疗结果及死亡率

Clinical Epidemiology, Treatment Outcome and Mortality Rate of Newly Diagnosed Immune Thrombocytopenia in Adult Multicentre Study in Malaysia.

作者信息

Hamzah Roszymah, Yusof Nurasyikin, Tumian Nor Rafeah, Abdul Aziz Suria, Mohammad Basri Nur Syahida, Leong Tze Shin, Ho Kim Wah, Selvaratnam Veena, Tan Sen Mui, Muhamad Jamil Siti Afiqah

机构信息

Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia.

Department of Haematology, Ampang Hospital, Ampang, Selangor, Malaysia.

出版信息

J Blood Med. 2022 Jun 21;13:337-349. doi: 10.2147/JBM.S358993. eCollection 2022.

Abstract

BACKGROUND

Immune thrombocytopenia (ITP) is well characterized in Western, European and other Asia-Pacific countries. Nevertheless, the clinical epidemiology, treatment pattern and disease outcome of ITP in Malaysia are still limited and not well known.

OBJECTIVE

This study aimed to describe the clinical epidemiology, treatment outcome and mortality of ITP patients in haematology tertiary multicentre in Malaysia.

METHODS

Clinical and laboratory data of newly diagnosed adults with ITP by a platelet count <100 × 10/L from January 2010 to December 2020 were identified and analyzed.

RESULTS

Out of 500 incident ITP, 71.8% were females with a striking age preponderance of both genders among those aged 18-29 years. The median age was 36 years. The median platelet count was 17.5 × 10/L, 23.0% had a secondary ITP, 34.6% had a Charlson's score ≥1, 53.0% had bleeding symptoms including 2.2% intracranial bleedings (ICB). screening was performed in <5% of cases. Persistency and chronicity rates were 13.6% and 41.8%, respectively. Most (80.6%) were treated at diagnosis onset and 31.2% needed second-line treatment. Throughout the course of ITP, 11.0% of patients died; 3.0% and 8.0% with bleeding and non-bleeding related ITP.

CONCLUSION

This study confirms the epidemiology of ITP is comparable with worldwide studies. Our incidence is high in the female, Malay ethnicity, primary ITP and events of cutaneous bleeding at ITP onset with 18-29 years predominance age group for both genders. The frequency of persistent and chronic ITP is inconsistent with published literature. Corticosteroids and immunotherapies are the most prescribed first-line and second-line pharmacological treatments. Thrombopoietin receptor agonist medications (TPO-RAs) usage is restricted and splenectomy is uncommon. Our mortality rate is similar but ITP related bleeding death is fourth-fold lower than previous studies. Mortality risks of our ITP patients include age ≥60 years, male, severe bleeding at presentation, CCI≥1 and secondary ITP.

摘要

背景

免疫性血小板减少症(ITP)在西方国家、欧洲及其他亚太国家已有充分的特征描述。然而,马来西亚ITP的临床流行病学、治疗模式和疾病转归仍然有限且鲜为人知。

目的

本研究旨在描述马来西亚血液学三级多中心ITP患者的临床流行病学、治疗结局和死亡率。

方法

确定并分析了2010年1月至2020年12月期间新诊断的血小板计数<100×10⁹/L的成年ITP患者的临床和实验室数据。

结果

在500例初发ITP患者中,71.8%为女性,18至29岁年龄组中两性的年龄优势明显。中位年龄为36岁。中位血小板计数为17.5×10⁹/L,23.0%患有继发性ITP,34.6%的查尔森评分≥1,53.0%有出血症状,包括2.2%的颅内出血(ICB)。<5%的病例进行了筛查。持续性和慢性率分别为13.6%和41.8%。大多数(80.6%)在诊断开始时接受治疗,31.2%需要二线治疗。在ITP的整个病程中,11.0%的患者死亡;3.0%和8.0%分别死于出血相关和非出血相关的ITP。

结论

本研究证实ITP的流行病学与全球研究相当。我们的发病率在女性、马来族、原发性ITP以及ITP发病时皮肤出血事件中较高,18至29岁年龄组在两性中占优势。持续性和慢性ITP的频率与已发表的文献不一致。皮质类固醇和免疫疗法是最常用的一线和二线药物治疗。血小板生成素受体激动剂药物(TPO-RAs)的使用受限,脾切除术不常见。我们的死亡率相似,但ITP相关出血死亡比以前的研究低四倍。我们ITP患者的死亡风险包括年龄≥60岁、男性、就诊时严重出血、CCI≥1和继发性ITP。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/013c/9233512/46d84b8e433c/JBM-13-337-g0001.jpg

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