Karti S, Yilmaz M, Sonmez M, Akdogan R, Ersoz S, Ucar F, Ovali E
Dept. of Internal Medicine, Division of Hematology, Karadeniz Technical University, School of Medicine, Trabzon, Turkey.
J Exp Clin Cancer Res. 2003 Jun;22(2):341-2.
Pure red cell aplasia (PRCA) is a rare disorder which is associated with thymoma, viral infections and autoimmune diseases. A few cases of PRCA during the clinical course of CML have been reported and these usually terminate in blastic crisis and death, suggesting a poor prognosis. However, only one case of Philedelphia chromosome negative, Bcr-Abl positive CML associated with PRCA has been reported. Here, we present a second case report of a Philedelphia negative, Bcr-Abl positive CML associated with PRCA who was unresponsive to all the chemotherapeutic regimens. We conclude that the present case supports the idea that the development of PRCA in the course of CML may be a bad prognostic sign.
纯红细胞再生障碍性贫血（PRCA）是一种罕见的疾病，与胸腺瘤、病毒感染和自身免疫性疾病有关。已有报道称，在慢性粒细胞白血病（CML）的临床过程中出现了少数PRCA病例，这些病例通常会发展为急变期并导致死亡，提示预后不良。然而，仅报道过1例费城染色体阴性、Bcr-Abl阳性的CML合并PRCA的病例。在此，我们报告第2例费城染色体阴性、Bcr-Abl阳性的CML合并PRCA的病例，该患者对所有化疗方案均无反应。我们得出结论，本病例支持以下观点：CML病程中出现PRCA可能是预后不良的征兆。
