儿童获得性低巨核细胞性血小板减少性紫癜临床特点分析

Analysis of clinical characteristics in children with aquired amega karyocytic thrombocytopenic purpura

目的通过分析儿童获得性低巨核细胞性血小板减少性紫癜（acquired amegakaryocytic thrombocytopenic purpura，AATP）的临床资料，疏理其临床特点，为临床准确诊断治疗提供帮助。方法回顾性分析北京儿童医院血液病中心2005年至2006年收治的9例AATP病人的临床资料。结果男女性别发病比例无明显差异，临床均以出血为主要症状和体征；患儿骨髓髂后0～5个／片活检未见巨核细胞。入院前院外诊断“特发血小板减少性紫癜”均治疗无效。入院后达到完全治疗反应者3例，部分有效2例，基本无效1例，无效1例，总有效率71．4％（5／7）；2例失访；3例转为再生障碍性贫血。结论儿童AATP临床仅表现与血小板减少相关的出血症状，骨髓穿刺及活检提示巨核细胞极度减少，而粒、红系统不受影响；大剂量丙种球蛋白和糖皮质激素无效，需要选择环孢茵素或抗胸腺球蛋白等T细胞免疫抑制剂治疗或骨髓移植；有42．

Objective According to analyzing clinical data of AATP, we had looked for clinical characteristic to provide assistance for correct diagnosis and therapy of clinic. Methods to review analysis clinical data of patients who admission to Beijing children＇ s hospital from 2005 to 2006. Results （ 1 ） general state ： age of onset of both sexes were similar, meso-age was 6.5 years old ; （2） clinicai state ： major symptom and physical sign was both hemorrhage; Megakaryocyte account of all of patients＇ bone marrow was 5 - 22/smear of sternum ,0 - 5/smear of ilium, none of biopsy of bone marrow ; （ 3 ） Therapy and prognosis ： all of patient were misdiagnosed ITP before admission, it was ineffective which apply high dose gammaglobulin and corticosteroids. After admission 7 cases were applied by Cyclosporine A（CsA） , 2 cases missed follow-up. 3 cases of 7 achieved complete response, 2 cases of 7 achieved partial response, 1 case was nearly ineffective, 1 case was ineffective. Effective power was 71.4% ; prognosis： 2 cases missed follow-up, 3 cases converted to aplastic anemia. Conclusion age of onset of both sexes were similar in children, pre-school and school age were common; unique symptom was hemorrhage due to thrombocytopenia, aspiration and biopsy of bone marrow discovered amegakaryocytic, leucocytes anderythrocytes were common; high dose gammaglobulin and corticosteroids were ineffective, necessary choices of therapy were immunosuppressive agent of T cell incruding CSA and ATG or bone marrow transplantation. Conversion of aplastic anemia was high.