Thrombotic microangiopathy

Question 1

What is Thrombotic Microangiopathy (TMA)?

Answer 1

Clinical syndromes characterized by hemolytic anemia, low platelets, and organ damage due to microscopic blood clots in capillaries and small arteries.

The kidneys are commonly affected, but any organ may be involved.

Question 2

What are the key features of TMA under the microscope?

Answer 2

Under the microscope, TMA shows injured red blood cells known as schistocytes or fragments.

Question 3

What are common causes of Thrombotic Microangiopathy?

Answer 3

thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), infections, medications, connective tissue diseases, cancer, vasculitis, pregnancy, malignant hypertension, organ transplant, and metabolic disorders.

Question 4

What causes Thrombotic Thrombocytopenic Purpura (TTP)?

Answer 4

TTP is caused by low activity of a protein called ADAMTS13, which can result from either a genetic mutation or acquired auto-antibodies that block its activity.

Question 5

How is Thrombotic Microangiopathy diagnosed?

Answer 5

TMA is diagnosed with blood tests that confirm red blood cell destruction, the presence of schistocytes, and organ damage. Specialized blood and genetic tests help identify the specific cause.

Question 6

What are typical organ damages associated with TMA?

Answer 6

Typical organ damage includes malignant hypertension, kidney injury, abdominal pain, diarrhea, stroke, confusion, heart injury, and eye damage.

Question 7

What is the treatment for Thrombotic Microangiopathy?

Answer 7

1. therapeutic plasma exchange (for TTP),
2. immune suppression,
3. complement system inhibitors (for aHUS),
4. antibiotics and supportive care for infections, and
5. dialysis for severe kidney injury.