获得性纯红细胞再生障碍性贫血的发病率：日本 2 个登记数据库的全国流行病学分析,Blood Advances

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获得性纯红细胞再生障碍性贫血的发病率：日本 2 个登记数据库的全国流行病学分析
Blood Advances ( IF 7.4 ) Pub Date : 2022-05-06 , DOI: 10.1182/bloodadvances.2021006486
Hideyuki Nakazawa ₁ , Kaoko Sakai ₁ , Akiko Ohta ₂ , Naohito Fujishima ₃ , Akira Matsuda ₄ , Kohei Hosokawa ₅ , Fumi Nakamura ₆ , Shinji Nakao ₅ , Kinuko Mitani ₆ , Fumihiro Ishida _{1,

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Affiliation

摘要获得性纯红细胞再生障碍性贫血（PRCA）是一种罕见的综合征，其特征是贫血伴网织红细胞减少和红细胞前体细胞显着减少。鉴于其罕见性，真实发病率在很大程度上是未知的，并且代表一般人群的流行病学数据以及对全部病因学的描述也很少。日本30年前进行的PRCA流行病学研究估计其年发病率为百万分之0.3。为了更新数据并调查 PRCA 的发病率和人口统计数据，我们利用日本血液学会 (JSH) 血液疾病登记处（由 JSH 管理的血液疾病登记数据库）和诊断程序组合 (DPC) 研究进行了一项全国范围内的流行病学研究数据可在日本厚生劳动省 (MHLW) 网站上获取。 2012 年至 2019 年间，共识别出 1055 名新诊断获得性 PRCA 患者，年平均发病率为 1.06（95% 置信区间 [CI]，0.83-1.28）/百万。中位年龄为 73 岁（范围为 18-99 岁）。女性与男性的比例为1.5：1，育龄组中女性优势最为突出。 69% 的获得性 PRCA 是特发性的。同期PRCA的发病率约为再生障碍性贫血（AA）的20%。每年每百万人中约有 0.98 名患者（95% CI，0.89-1.07）需要住院治疗 PRCA。这些结果预计将有助于讨论包括日本在内的许多国家人口老龄化中 PRCA 的资源分配问题。

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Incidence of acquired pure red cell aplasia: a nationwide epidemiologic analysis with 2 registry databases in Japan

Abstract Acquired pure red cell aplasia (PRCA) is a rare syndrome characterized by anemia with reticulocytopenia and a marked reduction in erythroid precursors. Given its rarity, the true incidence is largely unknown, and epidemiological data representing the general population, with a description of the full spectrum of etiologies, are scarce. An epidemiological study on PRCA in Japan conducted 30 years ago estimated the annual incidence as 0.3 per million. To update the data and investigate the incidence and demographics of PRCA, we conducted a nationwide epidemiological study using the Japanese Society of Hematology (JSH) Hematologic Disease Registry, a hematologic disease registration database managed by the JSH and the Diagnosis Procedure Combination (DPC) study data available at a website of the Ministry of Health, Labor, and Welfare (MHLW) of Japan. A total of 1055 patients with newly diagnosed acquired PRCA were identified between 2012 and 2019, and the average annual incidence was calculated at 1.06 (95% confidence interval [CI], 0.83-1.28) per million. The median age was 73 (range, 18-99) years. The female-to-male ratio was 1.5:1, and the female predominance was most prominent in the child-bearing age group. Sixty-nine percent of acquired PRCA was idiopathic. The incidence of PRCA was approximately 20% of that of aplastic anemia (AA) during the same period. Approximately 0.98 patients per million per year (95% CI, 0.89-1.07) required hospitalization for the treatment of PRCA. These results are expected to contribute to the discussion of resource allocation for PRCA in the aging population in many countries, including Japan.

更新日期：2022-05-06

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