Solignac Marie
Presse Med. 2003 Sep 13;32(29):1378-80.
Designated by the acronym PRCA or the term erythroblastopenia, pure red cell aplasia is characterised by severe anaemia with reticulocytopenia. It may occur in acute form induced by infectious agents, following drug toxicity or transplantation of allogeneic haematopoietic cells, associated with autoimmune haemolytic anaemia. The chronic form is rarely constitutional but can be acquired and is usually associated with blood or idiopathic diseases. IMMUNOLOGICAL INHIBITION OF ERYTHROPOIESIS: Among the mechanisms responsible for PRCA is immunological erythropoiesis inhibition. This may be of lymphocyte T cell origin or due to the presence of antibodies in the patient's serum. Although observations of PRCA with presence of neutralising antierythropoietin antibodies in patient's serum have multiplied over the past 5 years, they still remain extremely rare. From a therapeutic point of view, they require withdrawal of epoetin and often the administration of immunosuppressors and transfusion for symptomatic treatment. GROWTH FACTORS: The role of growth factors in restoring aplastic anaemia appears to be only partial, at random and temporary.
纯红细胞再生障碍性贫血简称为PRCA或成红细胞减少症，其特征为严重贫血伴网织红细胞减少。它可能以急性形式出现，由感染因子诱发，或在药物毒性作用后、同种异体造血细胞移植后发生，也可能与自身免疫性溶血性贫血相关。慢性形式很少是先天性的，但可以后天获得，通常与血液疾病或特发性疾病有关。
导致PRCA的机制之一是红细胞生成的免疫抑制。这可能源于淋巴细胞T细胞，或由于患者血清中存在抗体。尽管在过去5年中，观察到患者血清中存在中和抗促红细胞生成素抗体的PRCA病例有所增加，但仍然极为罕见。从治疗角度来看，需要停用促红细胞生成素，通常还需要给予免疫抑制剂并进行输血以进行对症治疗。
生长因子在恢复再生障碍性贫血方面的作用似乎只是部分的、随机的和暂时的。
