How is the presentation of PNH?

• Episodic (paroxysmal) hemolysis when complement is activated by mild acidosis: - Respiratory acidosis during sleep (nocturnal) - Lactic acidosis during exercise • Can lead to IDA (iron deficiency anemia) from chronic blood loss • Hepatic vein – Hepatic vein thrombosis (Budd-Chiari syndrome) in the setting of a hemolytic episode • Inferior vena cava, portal and splenic veins: Thrombosis of other intra-abdominal vessels • Dermal veins – discrete areas of erythema, swelling, and pain or as a syndrome resembling purpura fulminans • Hemolysis: related to anemia/hemolysis such as smooth muscle dystonia, pulmonary hypertension, and renal insufficiency. • Thrombosis: is the leading cause of death in PNH. It is relatively rare as a presenting symptom but eventually occurs in up to 40 percent of patients, if not treated. • Pancytopenia: could overlap with bone marrow disorders; eg.aplastic anemia (AA) or occasionally MDS. Other factors include iron deficiency and hypersplenism