Villarrubia J, Pascual C, Pérez Vaquero M A, Escribano L, Bellas C, Orte J, Heinrichs B, Velasco J L
Servicio de Hematología, Hospital Ramón y Cajal, Madrid.
Med Clin (Barc). 1992 Feb 1;98(4):139-41.
Pure red cell aplasia is manifested by unregenerative anemia with intense erythroid hypoplasia in the bone marrow and absolute reticulocytopenia. Its association with chronic myeloproliferative syndrome (CMS) is infrequent. The appearance of both processes may or may not be simultaneous and accidental association cannot be ruled out. Two cases of pure red cell aplasia were studied; one associated to Philadelphia positive chronic chromosome myeloid leukemia and the other associated to atypical CMS. In the first case no response was obtained with prednisone alone or associated to cyclophosphamide. In the second patient treatment with danazol was not efficient, however, corticotherapy produced a positive and maintained response. Treatment of pure red cell aplasia associated to CMS must be the same as that employed when this disease is presented as isolated or associated to other processes.
纯红细胞再生障碍性贫血表现为骨髓中红细胞生成严重低下的非再生性贫血及绝对网织红细胞减少。其与慢性骨髓增殖性综合征（CMS）的关联并不常见。这两种病症的出现可能同时发生，也可能不同时发生，不能排除偶然关联。我们研究了两例纯红细胞再生障碍性贫血病例；一例与费城染色体阳性慢性髓性白血病相关，另一例与非典型CMS相关。在第一例中，单独使用泼尼松或联合环磷酰胺均未取得疗效。在第二例患者中，达那唑治疗无效，然而，皮质激素治疗产生了阳性且持续的反应。与CMS相关的纯红细胞再生障碍性贫血的治疗必须与该疾病单独出现或与其他病症相关时所采用的治疗方法相同。
