Hamerschlak Nelson, Maluf Eliane, Pasquini Ricardo, Eluf-Neto José, Moreira Frederico Rafael, Cavalcanti Alexandre Biasi, Okano Iria Ruriko, Falcão Roberto Passeto, Pita Marimília Teixeira, Loggetto Sandra Regina, Rosenfeld Luiz Gastão, Lorand-Metze Irene Gyongyvér Heidemarie
Instituto Israelita de Ensino e Pesquisa Albert Einstein, São Paulo, Brazil.
Sao Paulo Med J. 2005 May 2;123(3):101-4. doi: 10.1590/s1516-31802005000300002. Epub 2005 Jul 8.
Aplastic anemia and agranulocytosis are rare but life-threatening disorders, often caused by drugs and other environmental exposures. Reported incidence of these diseases seems to vary between different geographic regions, and few data on their incidence are available for Latin American countries. The aim of this work is to determine the incidence of agranulocytosis and aplastic anemia in Brazil.
Incidence study. Seven centers took part in the pilot phase, so as to represent all Brazilian regions.
Each center conducted an active search for new cases in a defined region by means of regular contacts with all hematologists, main clinical laboratories and clinicians in hospitals of the region.
74 patients with aplastic anemia and 16 with agranulocytosis were identified. Patients with agranulocytosis had a median age of 31 years (interquartile range, IQR: 12.5-48.2); 32.2% were male and 81.2% were white. The median age of aplastic anemia patients was 21 years (IQR 15.0-35.2); 62.2% were male, 50.0% were white and 39.2% mulatto. The incidence of agranulocytosis was estimated to be 0.5 cases per million individuals per year, ranging from 0.0 to 1.1 cases per million per year between regions. The incidence of aplastic anemia was 2.7 cases per million per year, ranging from 1.1 to 7.1 cases per million per year between regions.
Aplastic anemia and agranulocytosis are rare diseases in Brazil. However, there is considerable variability in their incidences between different regions.
再生障碍性贫血和粒细胞缺乏症虽罕见但危及生命，常由药物及其他环境暴露因素引起。这些疾病的报告发病率在不同地理区域间似乎存在差异，而拉丁美洲国家关于其发病率的数据较少。本研究旨在确定巴西粒细胞缺乏症和再生障碍性贫血的发病率。
发病率研究。七个中心参与了试点阶段，以代表巴西所有地区。
每个中心通过与该地区所有血液科医生、主要临床实验室及医院临床医生定期联系，在指定区域积极搜索新病例。
共识别出74例再生障碍性贫血患者和16例粒细胞缺乏症患者。粒细胞缺乏症患者的中位年龄为31岁（四分位间距，IQR：12.5 - 48.2）；32.2%为男性，81.2%为白人。再生障碍性贫血患者的中位年龄为21岁（IQR 15.0 - 35.2）；62.2%为男性，50.0%为白人，39.2%为混血人种。粒细胞缺乏症的发病率估计为每年每百万人口0.5例，各地区范围为每年每百万人口0.0至1.1例。再生障碍性贫血的发病率为每年每百万人口2.7例，各地区范围为每年每百万人口1.1至7.1例。
再生障碍性贫血和粒细胞缺乏症在巴西是罕见疾病。然而，不同地区之间其发病率存在相当大的差异。
