Sorigue Marc, Juncà Jordi, Orna Elisa, Romanic Nevena, Sarrate Edurne, Castellvi Jordi, Soler Montse, Rodríguez-Hernandez Ines, Feliu Evarist, Ruiz Susana
Hematology Laboratory (Division of Thrombosis and Hemostasis), ICO-Badalona, Hospital Germans Trias i Pujol, Josep Carreras Leukemia Research Institute, Universitat Autonoma de Barcelona, Ctra. Canyet s/n, 08916, Badalona, Spain.
Hematology Laboratory, ICO-Badalona, Hospital Germans Trias i Pujol, Josep Carreras Leukemia Research Institute, Universitat Autonoma de Barcelona, Badalona, Spain.
J Thromb Thrombolysis. 2017 Jul;44(1):63-66. doi: 10.1007/s11239-017-1502-4.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder associated with increased risk for thrombosis and reduced life expectancy. Retinal vein occlusion (RVO) is a frequent cause of vision loss but its relationship with PNH has not been studied systematically. Patients followed up for RVO in our ophthalmology department were screened for the presence of a PNH clone in peripheral blood by means of flow cytometry. The presence of other well-documented risk factors for RVO was also analyzed. In a series of 110 patients (54 males, median age of 67) we found no evidence of PNH. Most patients (97/110) had cardiovascular risk factors and/or hyperhomocysteinemia (67/110). Inherited thrombophilias were rare (three confirmed cases). Therefore, PNH does not appear to play a role in the development of RVO. However, this finding does not necessarily apply to young patients and/or those with no conventional risk factors for RVO, due to the low number of patients in these subgroups in our population.
阵发性睡眠性血红蛋白尿（PNH）是一种罕见疾病，与血栓形成风险增加和预期寿命缩短相关。视网膜静脉阻塞（RVO）是视力丧失的常见原因，但其与PNH的关系尚未得到系统研究。我们眼科对RVO患者进行随访，通过流式细胞术筛查外周血中PNH克隆的存在情况。还分析了其他有充分文献记载的RVO危险因素。在一系列110例患者（54例男性，中位年龄67岁）中，我们未发现PNH的证据。大多数患者（97/110）有心血管危险因素和/或高同型半胱氨酸血症（67/110）。遗传性血栓形成倾向罕见（3例确诊病例）。因此，PNH似乎在RVO的发生中不起作用。然而，由于我们人群中这些亚组的患者数量较少，这一发现不一定适用于年轻患者和/或无RVO传统危险因素的患者。
