Microangiopathic hemolytic anaemia (MAHA) is a component of all of the following, except:
Thrombotic thrombocytopenic purpura (TTP)
Hemolytic uremic syndrome (HUS)
Hemolysis with elevated liver enzymes and low platelet (HELLP) syndrome
Paroxysmal nocturnal hemoglobinuria
- Paroxysmal nocturnal hemoglobinuria (PNH): Results from acquired mutations in PIGA gene leading to deficiency of GPI-linked proteins in membrane of blood cells. Red cells deficient in GPI-linked factors are abnormally susceptible to lysis or injury by complement. This manifests as intravascular hemolysis, which is caused by the C5b-C9 membrane attack complex. The hemolysis is paroxysmal and nocturnal in only 25% of cases; chronic hemolysis without dramatic hemoglobinuria is more typical. Other options: - TTP : characterized by pentad of Microangiopathic haemolytic anemia; Thrombocytopenia; Fluctuating neurologic dysfunction; Renal abnormalities & Fever. - HUS is characterized by triad of acute renal failure, thrombocytopenia & microangiopathic hemolytic anemia. - HELLP syndrome : The main three diagnostic criteria include : Hepatic dysfunction thrombocytopenia microangiopathic hemolytic anaemia in patients suspected to have preeclampsia.
Hemolytic Anemias: Basic concepts and classification