Oliveira Deivide Sousa, Lima Tadeu G, Benevides Fernanda L Neri, Barbosa Suzanna A Tavares, Oliveira Maria A, Boris Natália P, Silva Herivaldo F
Hospital Geral Dr. César Cals (HGCC), Fortaleza, CE, Brazil.
Hospital Geral Dr. César Cals (HGCC), Fortaleza, CE, Brazil.
Hematol Transfus Cell Ther. 2019 Apr-Jun;41(2):119-124. doi: 10.1016/j.htct.2018.10.002. Epub 2019 Feb 18.
Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease that requires early diagnosis and treatment that can be made possible by applying the PLASMIC score. This study aims to evaluate this score applicability for patients with suspected TTP in a developing country.
This was a retrospective study performed at a tertiary hospital in the northeastern region of Brazil. Patients were analyzed in two groups: ADAMTS13 activity <10% and activity >10%. Patients were stratified according to the PLASMIC score, and the level of agreement between the PLASMIC score and the ADAMTS13 activity was evaluated.
Eight patients with thrombotic microangiopathy were included. Four patients had ADAMTS13 activity <10%, all with a PLASMIC score =6. The other four had ADAMTS13 activity >10%, all with a score <6. Based on a score =6 for presumptive diagnosis of TTP, we attained a 100% diagnostic accuracy in our sample. The PLASMIC score was also able to accurately predict response to plasma exchange and the risk of long-term unfavorable outcomes.
The reproducibility of the PLASMIC score was quite satisfactory in our sample. It accurately discriminates between patients who had ADAMTS13 deficiency and those with normal enzyme activity, precluding the need for specific laboratory evaluation, which is not always available. This score can be useful for an early diagnosis and indicates which patients will benefit from the treatment in developing countries.
血栓性血小板减少性紫癜（TTP）是一种潜在的致命疾病，需要早期诊断和治疗，而应用PLASMIC评分有助于实现这一点。本研究旨在评估该评分在一个发展中国家疑似TTP患者中的适用性。
这是一项在巴西东北部一家三级医院进行的回顾性研究。患者被分为两组：ADAMTS13活性<10%和活性>10%。根据PLASMIC评分对患者进行分层，并评估PLASMIC评分与ADAMTS13活性之间的一致性水平。
纳入了8例血栓性微血管病患者。4例患者的ADAMTS13活性<10%，所有患者的PLASMIC评分为6分。另外4例患者的ADAMTS13活性>10%，所有患者的评分<6分。基于TTP初步诊断评分为6分，我们在样本中获得了100%的诊断准确率。PLASMIC评分还能够准确预测血浆置换的反应以及长期不良结局的风险。
在我们的样本中，PLASMIC评分的可重复性相当令人满意。它能够准确区分ADAMTS13缺乏的患者和酶活性正常的患者，无需进行并非总能进行的特定实验室评估。该评分有助于早期诊断，并能指出哪些患者将在发展中国家从治疗中获益。
