) microangiopathic hemolytic anemia
(MAHA) is a microangiopathic
subgroup of hemolytic anemia
, loss of red blood cells through destruction) caused by factors in the small blood vessels. It is identified by the finding of anemia and schistocytes
of the blood film
In diseases such as hemolytic uremic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, as well as malignant hypertension, the endothelial layer of small vessels are damaged with resulting fibrin deposition and platelet aggregation. As red blood cells travel through these damaged vessels, they are fragmented resulting in intravascular hemolysis. The resulting schistocytes are also increasingly targeted for destruction by the reticuloendothelial system in the spleen.
Automated analysers (the machines that perform routine full blood counts in most hospitals) are generally programmed to flag blood films that display red blood cell fragments or schistocytes.
The most important causes are:
In all causes, the mechanism of MAHA is the formation of a fibrin
mesh due to increased activation of the system of coagulation
. The red blood cells are physically cut by these protein networks, and the fragments are identical to the schistocytes seen on light microscopy.