Zamora-Pérez Elia, López-Karpovitch Xavier
Departamento de Hematología y Oncología, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México, D.F.
Gac Med Mex. 2015 May-Jun;151(3):345-53.
Myelodysplastic syndromes (MDS) are clonal diseases of hematopoietic cells. The International Prognostic Scoring System (IPSS) is the risk scale most employed in MDS. Cyclosporin A (CsA) has been used in the treatment of cytopenias in MDS.
To evaluate hematologic response and identify response predictive factors in adults with MDS treated with CsA.
Patients with MDS diagnosed according World Health Organization (WHO) classification were recruited from January 1997 to June 2012. All patients were classified with IPSS, IPSS revised (IPSS-R),WHO Prognostic Scoring System (WPSS), and WPSS revised (WPSS-R) risk scales. Cyclosporin A was administered orally at a dose of 5 mg/kg/day. Hematologic response was evaluated following the International Working Group for MDS (2006 version) criteria.
Inclusion criteria were met by 32 patients. Median age was 56.5 years, with a median follow-up of 3.1 years. Hematologic response was 56.2% and erythrocyte independence transfusion was found in 42.9% of patients. Age,hemoglobin level, and WPSS at diagnosis were independent predictive factors for CsA response. Survival was longer in responder than in nonresponder CsA patients (p=0.06).
Cyclosporin A induced hematologic response in >50% of patients with MDS aged <57 years, with Hb<8 g/dl and low WPSS at diagnosis.
骨髓增生异常综合征（MDS）是造血细胞的克隆性疾病。国际预后评分系统（IPSS）是MDS中使用最广泛的风险评估量表。环孢素A（CsA）已被用于治疗MDS的血细胞减少症。
评估接受CsA治疗的成年MDS患者的血液学反应，并确定反应预测因素。
1997年1月至2012年6月招募了根据世界卫生组织（WHO）分类诊断为MDS的患者。所有患者均采用IPSS、修订的IPSS（IPSS-R）、WHO预后评分系统（WPSS）和修订的WPSS（WPSS-R）风险量表进行分类。环孢素A以5mg/kg/天的剂量口服给药。根据国际MDS工作组（2006版）标准评估血液学反应。
32例患者符合纳入标准。中位年龄为56.5岁，中位随访时间为3.1年。血液学反应率为56.2%，42.9%的患者实现了红细胞输注独立。年龄、血红蛋白水平和诊断时的WPSS是CsA反应的独立预测因素。CsA反应者的生存期比无反应者更长（p=0.06）。
环孢素A可使57岁以下、诊断时Hb<8g/dl且WPSS低的MDS患者中超过50%产生血液学反应。
