特发性血小板减少性紫癜262例

Two Hundred and Sixty Two Children with Idiopathic Thrombocytopenic Purpura

目的 分析儿童特发性血小板减少性紫癜（ITP）发病规律、治疗及预后因素。方法1．总结北京儿童医院血液病中心2002～2003年住院ITP病例。分型并记录性别、年龄、血小板数、出血程度、诊断和治疗结果，比较急性与慢性、难治性ITP病例资料。2．对病程达0．5年以上患儿发信随访。3．总结死亡病例资料。结果 ITP患儿262例，其中急性ITP235例（89．7％），慢性24例（9．2％），难治性3例（1．15％）。1．急性组〈6岁高发（81％），常有明显诱因，治疗恢复快。2.慢性、难治性组〉6岁较多（33％），无明显诱因，治疗有效率低，恢复时间长。3．发病多为血小板重度、极重度减少（83．5％），临床多为轻度出血（70．2％）。4．随访66例：急性ITP18例6个月后确诊为慢性ITP；2例随访时确诊为再生障碍性贫血；3例（10％）慢性／难治性ITP病程6～12个月自行缓解。5．死亡1例，为慢性型，长期服用激素造成肾上腺皮质危象死亡。结论 1．儿童ITP以急性为主，恢复快，预后好。2．部分慢性、难治性ITP患儿有自愈可能。3.切忌过度治疗。4．疗效不佳者需再评估。

Objective To explore causes, treatment and prognosis of childhood idiopathic thrombocytopenic purpura （ITP） by analyzing clinical data of the patients in Beijing Children＇s Hospital. Methods Two hundred and sixty two ITP cases were diagnosed and treated in Hematology Center of Beijing Children＇s Hospital during 2002-2003 were reviewed. Characteristics of childhood ITP, such as type, sex, age, platelet count and bleeding degrees were analyzed by reviewing medical records and following up. Results Among 262 cases, acute ITP,chronic ITP（CITP） and refractory ITP（RITP） accounted for 235 （89.7%） ,24 （9.2%） and 3 （ 1.15% ）, respectively. 1. Acute ITP occurred frequently in children less than 6 year old（81% ） ,with predominant causes and rapid remission. 2. For chronic/refractory ITP,33% of the patients were older than 6 year old with unclear causes and lower remission. 3. No significant difference between girl and boy; no peak-season; no significantly prodromic infection; the majority case of ITP had severe thrombocytopenia （ 83.5 % ）, but only with mild bleeding（70.2% ）. 4. In 66 cases followed-up, 18 of 19 cases who were initially diagnosed as acute ITP developed into CITP 6 months later, and another one developed into aplastic anemia （AA） ;3 patients （ 10 % ） whose diagnosis were CITP obtained remission during the period from 6 to 12 months. 5. Only 1 case died of adrenal crisis because of long-term glucocorticoid treatment. Conclusions 1. The majority of the children＇s ITP is acute course, and there are the kind of patients who will recover fast and prognosis will better. 2. Part of the CITP/RITP patients will obtain remission automatically. 3. Excessive treatment isn＇t be encouraged. 4. The reassessment is necessary for the no-recovered patients.