Kuwana M, Kurata Y, Fujimura K, Fujisawa K, Wada H, Nagasawa T, Nomura S, Kojima T, Yagi H, Ikeda Y
Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
J Thromb Haemost. 2006 Sep;4(9):1936-43. doi: 10.1111/j.1538-7836.2006.02091.x.
We proposed diagnostic criteria for immune thrombocytopenic purpura (ITP) by modifying the existing guidelines for diagnosis of ITP and by incorporating laboratory tests found useful for predicting its diagnosis, for example erythrocyte count, leukocyte count, anti-GPIIb/IIIa antibody-producing B cells, platelet-associated anti-GPIIb/IIIa antibodies, percentage of reticulated platelets, and plasma thrombopoietin.
To validate our criteria, we conducted a multi-center prospective study involving 112 patients with thrombocytopenia and a morphologically normal peripheral blood film at the first visit. Each patient underwent a physical examination, routine laboratory tests, and specialized tests for the anti-GPIIb/IIIa antibody response and platelet turnover.
Ninety-one patients (81%) satisfied the proposed criteria at first visit. Clinical diagnosis was made by skilled hematologists > 6 months after the first visit; ITP was diagnosed in 88 patients and non-ITP disorders in 24. The proposed criteria had 98% sensitivity, 79% specificity, a 95% positive predictive value, and a 90% negative predictive value. A relatively low specificity appears to be attributed to a few patients who had both ITP and aplastic anemia or myelodysplastic syndrome.
Our preliminary diagnostic criteria based on ITP-associated laboratory findings were useful for the differential diagnosis of ITP, but additional evaluations and modifications will be necessary to develop criteria that can be used routinely.
我们通过修改现有的免疫性血小板减少性紫癜（ITP）诊断指南，并纳入对预测其诊断有用的实验室检查，如红细胞计数、白细胞计数、产生抗GPIIb/IIIa抗体的B细胞、血小板相关抗GPIIb/IIIa抗体、网织血小板百分比和血浆血小板生成素，提出了ITP的诊断标准。
为验证我们的标准，我们进行了一项多中心前瞻性研究，纳入了112例初诊时血小板减少且外周血涂片形态正常的患者。每位患者均接受了体格检查、常规实验室检查以及针对抗GPIIb/IIIa抗体反应和血小板更新的专门检查。
91例患者（81%）初诊时符合提出的标准。临床诊断由经验丰富的血液科医生在初诊6个月后做出；88例诊断为ITP，24例诊断为非ITP疾病。提出的标准敏感性为98%，特异性为79%，阳性预测值为95%，阴性预测值为90%。相对较低的特异性似乎归因于少数同时患有ITP和再生障碍性贫血或骨髓增生异常综合征的患者。
我们基于ITP相关实验室检查结果的初步诊断标准对ITP的鉴别诊断有用，但需要进一步评估和修改以制定可常规使用的标准。
