Koyama R V, Silva L F, Henriques V B, Tran C, Yoshikawa G T
Núcleo de Medicina Tropical do Pará
Universidade do Estado do Pará
Acta Reumatol Port. 2014 Jul-Sep;39(3):265-8.
Pure red cell aplasia is a rare condition described in patients with autoimmune diseases such as systemic lupus erythematosus. Bone marrow examination of a 52-year-old female showed selective severe hypoplasia, scarce hematopoietic reserves, and no abnormality in other cell lineages, which are findings compatible with red cell aplasia. This condition has not responded to corticosteroids, cytotoxic drugs or intravenous immunoglobulin. After therapy with high doses of glucocorticoids, cyclophosphamide, and immunoglobulin failed, she was treated with human recombinant erythropoietin, monthly pulses of methylprednisolone, and cyclophosphamide, simultaneously. Data on treatment with erythropoietin for pure red cell aplasia associated with systemic lupus erythematosus is limited, but it appears to be reasonable to try in refractory cases.
纯红细胞再生障碍性贫血是一种在系统性红斑狼疮等自身免疫性疾病患者中描述的罕见病症。一名52岁女性的骨髓检查显示选择性严重发育不全、造血储备稀少，且其他细胞系无异常，这些发现与红细胞再生障碍相符。这种情况对皮质类固醇、细胞毒性药物或静脉注射免疫球蛋白均无反应。在高剂量糖皮质激素、环磷酰胺和免疫球蛋白治疗失败后，她同时接受了重组人促红细胞生成素、甲泼尼龙每月脉冲治疗和环磷酰胺治疗。关于促红细胞生成素治疗与系统性红斑狼疮相关的纯红细胞再生障碍性贫血的数据有限，但在难治性病例中尝试似乎是合理的。
