一例异基因外周血干细胞移植致Ⅳ度肠道aGVHD的临床研究  

Clinical Study on A Patient with Intestinal Acute Graft-Versus-Host Disease Grade Ⅳ after Allo-Peripheral Blood Stem Cell Transplantation

作　　者：陈纯[1] 方建培[1] 黄绍良[1] 包蓉[1] 吴燕峰[1] 

机构地区：[1]中山医科大学孙逸仙纪念医院儿科,广州510120

出　　处：《中国实验血液学杂志》2001年第3期228-231,共4页Journal of Experimental Hematology

摘　　要：对 1例异基因外周血干细胞移植 (allo PBSCT)治疗重型 β地中海贫血 (TM )患儿合并Ⅳ度肠道aGVHD的诊断和治疗进行分析和探讨。预处理方案为白消安 (18mg/kg) ,环磷酰胺 (2 0 0mg/kg) ,噻替派 (6mg/kg) ,马抗胸腺细胞球蛋白 (110mg/kg)。输注的NC为 10 .6× 10 8/kg(受体体重 ,下同 ) ,CD34+ CD38- 11.7× 10 6 /kg。PBSCT + 14天WBC为 1.4× 10 9/L ,ANC为 0 .66×10 9/L ,植入证据为“供者型” ,但WBC在 1.4 - 2 .5× 10 9/L ,呈“再障”骨髓象 ,于 + 39天予输注供体细胞后 ,逐渐恢复正常造血。患儿 + 11天发生了皮肤Ⅱ度aGVHD ,在其好转时 + 33天出现腹泻 ,于 + 39天第二次输注供体细胞后明显加重 ,持续 2个月 ,最终按肠道aGVHD治疗有效。本例表明在皮肤aGVHD好转后仍可发生严重的肠道aGVHD ;allo PBSCT时对于顽固性腹泻的患儿 ,如植入证据明确 ,但外周血象和骨髓象呈抑制状态时 ,应考虑肠道aGVHD。The purpose of this study was to designed to analyze and explore the diagnosis and treatment on a patient with β-thalassemia major accompanied with intestinal acute graft-versus-host disease(aGVHD) grade Ⅳ after allo-peripheral blood stem cell transplantation(allo-PBSCT). The conditioning regimen was consisted of busulfan(18 mg/kg), cyclophosphamide(200 mg/kg), thiotepa(6 mg/kg) and horse anti-thymocyte globulin(110 mg/kg). The nucleated cells (10.6×10 9/kg) and CD34 +CD38 - cells (11.7×10 6/kg) were infused. At +14 days PBSCT, ANC and WBC achieved 0.66×10 9/L and 1.4×10 9/L respectively. The engraftment was successful but the patient depended on transfusion. The range of blood WBC was from 1.4×10 9/L to 2.4×10 9/L. The myelogram showed aplastic anemia. Hematopoiesis was restored continually after the second transfusion of the donor′s cells. At +11 days the patient was suffered from cutaneous aGVHD grade Ⅱ. Diarrhea occurred at +33 day when cutaneous aGVHD had been improved, and exaggerated obviously after the second transfusion at +39 days. The serious diarrhea continued for 2 months and intestinal aGVHD grade Ⅳ was finally diagnosed. Our conclusion was that intestinal aGVHD possibly occurred even when cutaneous aGVHD was improved. For a patient with refractory diarrhea after allo-PBSCT, intestinal aGVHD was considered first when the engraftment evidence was definited but peripheral blood cells and bone marrow were abnormal.

关 键 词：异基因外周血干细胞移植 急性移植物抗宿主病 肠道移植物抗宿主病 重型Β地中海贫血 TM ALLO-PBSCT 

分 类 号：R457.7[医药卫生—治疗学] R556.61[医药卫生—临床医学]