PNH is a Complement mediated intravascular blood cell lysis which is caused by the mutation of Phosphatidylinositol glycan A (PIG-A) gene. A membrane anchor lipid called Glycophosphatidylinositol (GPI) is coded by PIG-A gene, which is responsible to bind with several other proteins like decay accelerating factor (DAF), CD59 etc. These kinds of anchoring, inhibit the action of cell lysis by complement system and keep the necessary body cells safe from the immune and complement system attack. Now if there is any problem is the synthesis of GPI due to PIG-A mutation/ inactivation then those anchoring activities will be interrupted and complement system will accelerate the lysis (called membrane attack complex-MAC) and the blood cells will destructed. Even though the name suggest that the disease is acute in nature, it will occur at night and there will be hemolysis but it is chronic in nature, it can occur any time and it is not related to hemolysis, PIG-A mutation also can cause leucopenia and thrombocytopenia. This paper will elaborate the detailed pathology of the diseases and how the MAC could be inhibited using a monoclonal antibody.

S. Kudiyarasan earned a B.E. degree in electrical and electronics engineering from Bharathiar University in Coimbatore, India, in 2002, an M.E. degree in applied electronics from Anna University in Chennai, India, in 2005, and a Ph.D. degree in electrical and electronics engineering from Academy of Maritime Education and Training (AMET) University in Kanathur, India, in 2019. He is currently a Scientific Officer for the Department of Atomic Energy's 500 MW Prototype Fast Breeder Nuclear Reactor Project in India. He is also working on industrial-scale dissimilar joints using the Magnetic Pulse Welding technology. His research focuses on power electronics, bio medical electronics and electric drives. He has also published scientific papers in a variety of venues, including Elsevier, Springer, and Wiley. He has also authored IEEE Transactions and a few book chapters. He holds scientific membership titles such as Professional Engineer (India), Charted Engineer (India), and Fellow (IEI, India).