Centro de Hematología y Medicina Interna de Puebla, Puebla, México.
3972Benemérita Universidad Autónoma de Puebla, Puebla, México.
Clin Appl Thromb Hemost. 2021 Jan-Dec;27:10760296211044212. doi: 10.1177/10760296211044212.
The sticky platelet syndrome (SPS) was described by Mammen in 1983. Since then, scientists in several countries have identified the condition and published cases or series of patients, thus enabling the description of the prevalence of the inherited condition, its salient clinical features, and the treatment of the disease. The diagnosis of the SPS phenotype requires fresh blood samples and special equipment which is not available in all coagulation laboratories. In the era of molecular biology, up to now it has not been possible to define a clear association of the SPS phenotype with a specific molecular marker. Some molecular changes which have been described in platelet proteins in some persons with the phenotype of the SPS are here discussed. Nowadays, the SPS phenotype may be considered as a risk factor for thrombosis and most cases of the SPS developing vaso-occlussive episodes are the result of its coexistence with other thrombosis-prone conditions, some of the inherited and some of them acquired, thus leading to the concept of multifactorial thrombophilia. Ignoring all these evidence-based concepts is inappropriate, same as stating that the SPS is a nonentity simply because not all laboratories are endowed with adequate equipment to support the diagnosis.
黏附血小板综合征（SPS）于 1983 年由 Mammen 描述。此后，多个国家的科学家已经确定了这种病症，并发表了病例或一系列患者，从而能够描述遗传病症的流行情况、其显著的临床特征和疾病的治疗方法。SPS 表型的诊断需要新鲜的血液样本和并非所有凝血实验室都具备的特殊设备。在分子生物学时代，迄今为止，还不可能将 SPS 表型与特定的分子标志物明确联系起来。本文讨论了一些在具有 SPS 表型的人的血小板蛋白中描述的分子变化。如今，SPS 表型可能被视为血栓形成的危险因素，大多数发生血管阻塞性发作的 SPS 病例是其与其他易栓症并存的结果，其中一些是遗传性的，另一些是获得性的，从而导致了多因素易栓症的概念。忽视所有这些基于证据的概念是不合适的，同样，仅仅因为并非所有实验室都具备支持诊断的足够设备就断言 SPS 不存在也是不合适的。
