![]() The complement system is part of the innate immune system that has three main activation pathways: Classical, alternative, and lectin-mediated. The main pathogenetic mechanism that mediates organ damage and mortality is the dysregulation of the complement system. Other severe complications include renal failure, pulmonary hypertension, peripheral neuropathy and smooth muscle dystonia, which can manifest as chest pain, abdominal pain, dysphagia, and erectile dysfunction. , The primary causes of death in PNH patients are thrombotic complications, which account for 40-67% of fatal outcomes. Evidence from major epidemiological studies shows that despite up-to-date management, 35% of patients with classical PNH die within the first 5-year of diagnosis, and the 10-year mortality rate is about 50%. PNH is associated with a significant increase in mortality, development of arterial and venous thrombosis, visceral organ damage, and rapid deterioration in quality of life. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, rare, life-threatening, progressive systemic disorder, characterized by chronic intravascular hemolysis. Paroxysmal nocturnal hemoglobinuria: Diagnosis and management protocol. How to cite this URL: Almomen AK, Al Bakistani AG, Alsaeed A, Al Olama A, Hejazi A, Awarji C, Almhareb F, Alsayegh F, Alzahrani H, Almarashly M, Qari M, Aslam M, Seliem R, Al Kindi S, Abuharbesh S, Owaidah T, Bassuni W. How to cite this article: Almomen AK, Al Bakistani AG, Alsaeed A, Al Olama A, Hejazi A, Awarji C, Almhareb F, Alsayegh F, Alzahrani H, Almarashly M, Qari M, Aslam M, Seliem R, Al Kindi S, Abuharbesh S, Owaidah T, Bassuni W. Keywords: Epidemiological studies, paroxysmal, phosphatidylinositol This information reflects their experience and to assist specialists looking after PNH patients, including hematologists, nephrologists, dialysis specialists, gastroenterologists, cardiologists, and surgeons. These recommendations are formulated by a panel of experts from the gulf cooperation countries. However, the heterogeneity of clinical symptoms and rarity of this disease usually results in untimely diagnosis, severe disability of patients, and increased risk of fatal complication. Early diagnosis of PNH is crucial for effective disease management. PNH etiology is an Υ-linked gene somatic mutation of the phosphatidylinositol glycan class ΐ ( PIG-A ), that results in deficiency of the glycosylphosphatidylinositol anchor structure responsible for fixing a wide spectrum of proteins on blood cell membranes, absence of these proteins, particularly CD55 and CD59, dysregulates the complement on cell membranes and results in significant chronic complement-mediated hemolysis. ![]() ![]() Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired, rare clonal blood disorder, characterized by chronic intravascular hemolysis, bone marrow failure, renal failure and pulmonary hypertension, and a heightened risk of thrombotic complications. Source of Support: None, Conflict of Interest: None Paroxysmal nocturnal hemoglobinuria: Diagnosis and management protocolĪbdul Kareem Almomen 1, Abdul Ghani Al Bakistani 2, Ahmad Alsaeed 3, Asma Al Olama 4, Ayman Hejazi 5, Christian Awarji 6, Fahed Almhareb 7, Faisal Alsayegh 6, Hazzaa Alzahrani 7, Mahmoud Almarashly 8, Mohammad Qari 9, Mohammad Aslam 2, Rania Seliem 10, Salam Al Kindi 11, Saud Abuharbesh 12, TareK Owaidah 7, Wafaa Bassuni 3ġ Department of Hematology and Oncology, King Saud University Medical City, Riyadh, Saudi Arabia 2 Departments of Hematology and Pathology, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia 3 Departments of Oncology, King Abdulaziz Medical City, Jeddah, Saudi Arabia 4 Department of Hematology, Dubai Hospital, Dubai, Saudi Arabia 5 Department of Oncology, King Abdulaziz Medical City, Riyadh, Saudi Arabia 6 Department of Pathology and Hematology, Mubarak Hospital, Kuwait City, Kuwait 7 Department of Hematology and Pathology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia 8 Department of Hematology, Dubai Hospital, Dubai, United Arab Emirates 9 Department of Hematology, College of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia 10 Department of Pathology, Rashid Hospital, Dubai, United Arab Emirates 11 Department of Hematology, Qabous University Hospital, Mascut, Oman 12 Department of Hematology, Security Forces Hospital, Riyadh, Saudi Arabia Date of Web PublicationĬorrespondence Address: Abdul Kareem AlmomenĬenter of Excellence in Thrombosis and Hemostasis, King Saud University Medical City, P. ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |