de Oliveira Leonardo Rodrigues, Ferreira Thaís Camargos, Neves Fernando de Freitas, Meneses Antônio Carlos de Oliveira
Universidade Federal do Triângulo Mineiro - UFTM, Uberaba, MG, Brazil.
Rev Bras Hematol Hemoter. 2013;35(5):366-8. doi: 10.5581/1516-8484.20130100.
Aplastic anemia is a bone marrow failure syndrome characterized by peripheral cytopenias and hypocellular bone marrow. Although aplastic anemia is idiopathic in most cases, rheumatic diseases such as systemic lupus erythematosus are recognized as causes of aplastic anemia, with their possible etiological mechanisms being T and B lymphocyte dysfunction and pro-inflammatory cytokines and autoantibody production directed against bone marrow components. In the course of the human immunodeficiency virus infection/acquired immunodeficiency syndrome, the identification of autoantibodies and the occurrence of rheumatic events, in addition to the natural course of systemic lupus erythematosus which is modified by immune changes that are characteristic of human immunodeficiency virus infection/acquired immunodeficiency syndrome, make the diagnosis of systemic lupus erythematosus challenging. This study reports the case of a woman with acquired immunodeficiency syndrome treated with a highly active antiretroviral therapy, who had prolonged cytopenias and hypocellular bone marrow consistent with aplastic anemia. The clinical picture, high autoantibodies titers, and sustained remission of the patient's hematological status through immunosuppression supported the diagnosis of systemic lupus erythematosus-associated aplastic anemia. This is the first report of aplastic anemia concurrent with systemic lupus erythematosus and acquired immunodeficiency syndrome, providing additional evidence that immune dysfunction is a key part of the pathophysiological mechanism of aplastic anemia.
再生障碍性贫血是一种骨髓衰竭综合征,其特征为外周血细胞减少和骨髓细胞减少。虽然大多数情况下再生障碍性贫血是特发性的,但风湿性疾病如系统性红斑狼疮被认为是再生障碍性贫血的病因,其可能的病因机制是T和B淋巴细胞功能障碍以及针对骨髓成分产生促炎细胞因子和自身抗体。在人类免疫缺陷病毒感染/获得性免疫缺陷综合征过程中,除了系统性红斑狼疮的自然病程会因人类免疫缺陷病毒感染/获得性免疫缺陷综合征的特征性免疫变化而改变外,自身抗体的识别和风湿性事件的发生也使得系统性红斑狼疮的诊断具有挑战性。本研究报告了一例接受高效抗逆转录病毒治疗的获得性免疫缺陷综合征女性患者,该患者出现了与再生障碍性贫血一致的持续性血细胞减少和骨髓细胞减少。临床表现、高滴度自身抗体以及通过免疫抑制患者血液学状态的持续缓解支持了系统性红斑狼疮相关再生障碍性贫血的诊断。这是首例再生障碍性贫血并发系统性红斑狼疮和获得性免疫缺陷综合征的报告,为免疫功能障碍是再生障碍性贫血病理生理机制的关键部分提供了额外证据。