Aplastic anemia associated with dyskeratosis congenita treated with antilymphocyte globulin and cyclosporine: a case report

作     者：Hsiu-MeiHuang Wen-LiangYu Yu-LunHuang Wei-ShiouHwang Chao-JungTsao Hsiao-ShengLiu Guan-ChengHuang 

作者机构：DepartmentofInternalMedicineChimeiMedicalCenterYungKangCityTainanTaiwan710China DepartmentofOphthalmologyChangGungMemorialHospitalKaohsiungTaiwan833China CollegeofMedicineNationalChengKungUniversityTainanTaiwan710China 

出 版 物：《Chinese Medical Journal》 (中华医学杂志（英文版）)

年 卷 期：2005年第118卷第9期

页      面：790-792页

核心收录：

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

主　　题：dyskeratosis congenita aplastic anemia antilymphocyte globulin immunosuppression therapy 

摘      要：Dyskeratosis congenita (DC) is a severe inherited disease characterized by a triad of clinical manifestations including abnormal skin pigmentation, nail dystrophy, and mucosal leukoplakia. 1 Bone marrow failure is the principal cause of early mortality, together with an increased predisposition to malignancy and fatal pulmonary complications. According to the dyskeratosis congenita registry, a peripheral blood cytopenia of one or more lineages is reported in 93% of patients, with 51% developing pancytopenia before the age of 10 years. 2 In patients with DC, bone marrow failure or bone marrow failure treatment-associated complications account for 67% of total mortality. 3 Therefore, management of bone marrow failure syndrome is crucial in patients with DC.