What is thrombotic microangiopathy?

Thrombotic microangiopathies (TMA) are clinical syndromes defined by the presence of hemolytic anemia (destruction of red blood cells), low platelets, and organ damage due to the formation of microscopic blood clots in capillaries and small arteries.

What are the symptoms of thrombotic microangiopathy?

Signs and symptoms

The clinical presentation of TMA, although dependent on the type, typically includes: fever, microangiopathic hemolytic anemia (see schistocytes in a blood smear), kidney failure, thrombocytopenia and neurological manifestations.

Is thrombotic microangiopathy curable?

What are the chances that I will get better? TTP was once fatal in 90% of individuals who developed the disease. Now that plasma exchange is available, survival can be as high as 80%. In many cases the blood vessel damage in the kidneys and brain will reverse with time.

How common is thrombotic microangiopathy?

The incidence of TTP in adults is about 3 per 1 000 000,2 and the incidence of HUS in children is about 3 per 100 000. Regardless of the etiology, thrombotic microangiopathy is a hematologic emergency that requires prompt treatment.

How is thrombotic microangiopathy diagnosed?

Because of the important role of the von Willebrand factor (vWF)–cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13), TTP is diagnosed by detection of thrombotic microangiopathy—that is, the presence of schistocytes (red blood cell fragmentation) in blood,

How is thrombotic microangiopathy treated?

For aHUS, patients are treated with an intravenous medication that blocks the complement system. For other diseases that cause TMA, the treatment focuses on managing the underlying disease. For example, infectious causes of TMA might be treated with antibiotics and supportive care.

When should you suspect thrombotic microangiopathy?

Thrombotic microangiopathy (TMA) is suspected in patients presenting with thrombocytopenia and evidence of a microangiopathic hemolytic anemia. Patients with TMA can be critically ill, so rapid and accurate identification of the underlying etiology is essential.