Abstract
Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal haematopoietic stem cell disease that presents with haemolytic anaemia, thrombosis and bone marrow failure. We report a case of a 51-year-old male with a history of PNH in treatment with Eculizumab admitted to our Hospital for acute chest pain and dyspnoea. The diagnosis was a triple vessel disease and patient was scheduled for coronary artery bypass grafting surgery. To balance the risk between thrombosis and bleeding in this particular clinical setting, we decided to use thromboelastography (TEG) as point of care solution and we used the R parameter as the target of our anticoagulant therapy. The R parameter between 11 and 14 sec can be used as a target value to balance the risk; in addition, there was no evidence of acute hemolysis during the surgery and supplemental dose of Eculizumab was administered in order to minimize any potential exacerbation of intravascular hemolysis.
Highlights
Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal haematopoietic stem cell (HSC) disease that presents with haemolytic anaemia, thrombosis and bone marrow failure
PNH is caused by somatic mutations in PIGA, an X-inked gene responsible for the biosynthesis of glycosylphosphatidylinositol (GPI) anchors; PIGA mutations leads to a deficiency of GPI-anchored proteins, such as CD55 and CD59, which are both complement inhibitors [1]
Thrombosis is the most serious complication which accounts for 40–67% of deaths in PNH
Summary
Аортокоронарное шунтирование у пациента с пароксизмальной ночной гемоглобинурией (клиническое наблюдение). For citation: Angela Amendola, Gianluca Paternoster, Sara Pasquina Pascale, Roberta Nuccorini, Mauro D'Amora, Giuseppe Pittella, Riccardo D'Ascoli, Filippo Prestipino, Giampaolo Luzi, Michele Pizzuti. Пароксизмальная ночная гемоглобинурия (ПНГ) — клональное заболевание гемопоэтических стволовых клеток, которое проявляется гемолитической анемией, тромбозами и недостаточностью функции костного мозга. Чтобы избежать развития как тромбоза, так и кровотечения, в данном сложном клиническом случае, решили провести пациенту тромбоэластографическое исследование и использовать параметр R в качестве целевого при проведении антикоагулянтной терапии. Во время оперативного вмешательства не наблюдали острого гемолиза, а для уменьшения риска развития внутрисосудистого гемолиза дополнительно назначили экулизумаб. Ключевые слова: пароксизмальная ночная гемоглобинурия; экулизумаб; тромбоэластограмма.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
