Aboud Nasra, Depré Fabian, Salama Abdulgabar
Institute of Transfusion Medicine, Charité Unversitätsmedizin Berlin, Germany.
Institute of Transfusion Medicine, Charité Unversitätsmedizin Berlin, Germany; Department of Cardiology and Pneumology, Campus Benjamin Franklin, Charité Universitätsmedizin Berlin, Germany.
Transfus Med Hemother. 2017 Jan;44(1):23-28. doi: 10.1159/000449038. Epub 2016 Oct 18.
Dependent on the absence or presence of associated diseases, autoimmune thrombocytopenia (ITP) can be classified as primary or secondary form. The manifestation of the associated diseases is not temporally defined and may occur during observation. Thus the question which disease is the primary one remains unanswered.
All 386 patients included in this study were treated by a single primary physician between 1996 and 2015 at the Charité Berlin and met current ITP criteria. Medical records and investigations were reviewed to assess diseases associated with ITP.
Initially, the vast majority of patients presented with primary ITP (isolated disease). Based on our findings, ITP was found to be associated with other abnormalities in most cases. These abnormalities included: positive direct antiglobulin test in 49 of 386 tested patients (13%), affections of the thyroid gland in 41 of 386 tested patients (11%), infections in 30 (8%), solid malignancies in 20 (5%) and hematological malignancies in 10 patients (3%), as well as many other miscellaneous diseases. Moreover, of 160 patients who did not receive prior intravenous immunoglobulin treatment, 40 (25%) showed antibody deficiency.
In conclusion, the incidence of 'true' ITP as a primary disease is less common than has yet been suggested. Additionally, there is evidence that ITP itself predispose affected subjects toward development of other diseases.
根据是否存在相关疾病,自身免疫性血小板减少症(ITP)可分为原发性或继发性。相关疾病的表现没有时间上的界定,可能在观察期间出现。因此,哪种疾病是原发性的问题仍未得到解答。
本研究纳入的所有386例患者在1996年至2015年期间由柏林夏里特医院的一位主治医生进行治疗,并符合当前ITP标准。回顾病历和检查结果以评估与ITP相关的疾病。
最初,绝大多数患者表现为原发性ITP(孤立性疾病)。根据我们的研究结果,发现大多数情况下ITP与其他异常有关。这些异常包括:386例受检患者中有49例(13%)直接抗球蛋白试验阳性,386例受检患者中有41例(11%)甲状腺受累,30例(8%)感染,20例(5%)实体恶性肿瘤,10例患者(3%)血液系统恶性肿瘤,以及许多其他杂病。此外,在160例未接受过静脉注射免疫球蛋白治疗的患者中,40例(25%)存在抗体缺陷。
总之,作为原发性疾病的“真正”ITP的发病率比目前认为的要低。此外,有证据表明ITP本身使受影响的个体易患其他疾病。