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Thrombotic Thrombocytopenic Purpura (TTP):

(HIGH YIELD NOTES FOR |MBBS|NEETPG|NEXT|INICET|FMGE)

 

A bleeding disorder due to platelet microthrombi that block small blood vessels, leading to end-organ ischemia and dysfunction.

RBCs fragmented by contact with the microthrombi, leading to hemolysis (microangiopathic hemolytic anemia).

 

 

Presentation:

  1. Low platelet count
  2. Microangiopathic hemolytic anemia
  3. Neurologic changes (delirium, seizure, stroke)
  4. Impaired renal function
  5. Fever

 

 

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Diagnosis:

  • Presence of schistocytes (broken RBCs) on peripheral smear
  • Low platlet count
  • High creatinine
  • Nucleated RBCs are also often seen in the peripheral smear

 

HUS:

  1. renal failure
  2. hemolytic anemia
  3. low platelets.

Severe elevations in creatinine levels are more typical of HUS than of TTP.

 

Remember: In HUS ,antibiotics are contraindicated because they can increase the toxin by destruction of bacteria & worsen the patient condition.

 

Treatment:

  • Steroids to ↓ microthrombus formation
  • plasmapheresis for severe cases.
  • Platelet transfusion is contraindicated, as it often worsens the patient’s condition 2° to added platelet aggregation and microvascular thrombosis

 

So I hope you know that:

  • The 3 causes of microangiopathic hemolytic anemia are HUS, TTP, and DIC.
  • In TTP, bleeding is not caused by decreasing of clotting factors ,so PT & aPTT are Normal.

 

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Q. An 8-year-old girl presents to the ER with 2 days of fever, vomiting, bloody diarrhea, and irritability. She began feeling unwell after attending a classmate’s birthday party. Her labs reveal thrombocytopenia and an ↑ creatinine level. What is the diagnosis & best antibiotic therapy?

A. HUS is the most common cause of acute renal failure in children.

Supportive therapy includes IV fluids, BP control, blood transfusion, and, if necessary, dialysis.

Antibiotics are not indicated, as they are thought to ↓ expulsion of the toxin and may ↑ toxin from the destruction of bacteria.

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