Blood Unit and National Reference Center for Congenital and Bleeding Disorders, Laikon General Hospital, Athens, Greece
Research Article
Detection of Subclinical/Minor PNH Clone(S) in Patients with Thrombotic Events
Author(s): Ioanna Anastasopoulou*, Paraskevi Kotsis, Stilliani Kokori, George Lallas, Sofia Gkotsi, Olga Katsarou and Konstantinos Konstantopoulos
Background: Paroxysmal nocturnal hemoglobinuria is a rare acquired disorder of a pluripotent hematopoietic stem cell, caused by a PIG-A gene somatic mutation. Thrombosis occurs in 40% of PNH patients and consists a main cause of morbidity. More than one-third of thrombotic episodes are located in hepatic veins, the inferior vena cava and cerebral veins.
Study objective: To evaluate the presence and role of PNH subclinical/minor clone(s) in idiopathic VTE patients, with no known congenital or acquired thrombophilia.
Study design: 181 patients having experienced idiopathic thrombosis and 100 healthy controls were screened for PNH clone(s) presence by flow cytometry. Testing was performed on white blood cells using FLAER and on red blood cells using CD59. The study was focused on the presence of minor.. View more»