PNH or Paroxysmal Nocturnal Haemoglobinuria

PNH is a life threatening and very rare blood disorder characterized by chronic hemolysis also known as Marchiafava-Micheli syndrome. Paroxysmal Nocturnal Hemoglobinuria (PNH) is a chronic and life threatening blood disorder in which affected red blood cells (RBCs) are constantly destroyed.  It is an acquired genetic disorder, meaning it is not inherited but rather develops through random mutations in a specific gene. Some RBCs in patients with PNH are missing a protein that normally protects them from destruction by a part of the immune system called complement.  The ongoing destruction of RBCs, called chronic hemolysis, is the primary cause of the various symptoms that PNH patients experience.