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Concept
Microangiopathic hemolytic anemia
Summary
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 on microscopy of the blood film.
In diseases such as hemolytic uremic syndrome, disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and malignant hypertension, the endothelial layer of small vessels is 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 (red cell fragments) are also increasingly targeted for destruction by the reticuloendothelial system in the spleen, due to their narrow passage through obstructed vessel lumina. It is seen in systemic lupus erythematosus, where immune complexes aggregate with platelets, forming intravascular thrombi. Microangiopathic hemolytic anemia is also seen in cancer.
Microangiopathic hemolytic anemia may be suspected based on routine medical laboratory tests such as a CBC (complete blood cell count). Automated analysers (the machines that perform routine full blood counts in most hospitals) are designed to flag blood specimens that contain abnormal amounts of red blood cell fragments or schistocytes.
Disseminated intravascular coagulation
HELLP syndrome
Thrombotic thrombocytopenic purpura
Hemolytic uremic syndrome
Cancer
Malignant hypertension
Scleroderma renal crisis
Malfunctioning cardiac valves (called the "Waring Blender syndrome")
Kasabach–Merritt syndrome
Insertion of foreign bodies
Drugs (e.g. cancer chemotherapy)
others diseases: eclampsia, renal allograft rejection, paroxysmal nocturnal hemoglobinuria, scleroderma, and vasculitides such as polyarteritis nodosa and granulomatosis with polyangiitis, antiphospholipid syndrome
In all causes, the mechanism of MAHA is the formation of a fibrin mesh due to increased activation of the system of coagulation.
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