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Acquired Thrombotic Thrombocytopenic Purpura

Autoantibody mediated ADAMTS thirteen deficiency causing microangiopathic hemolysis

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Acquired Thrombotic Thrombocytopenic Purpura?

Acquired thrombotic thrombocytopenic purpura results from autoantibodies that inhibit the von Willebrand factor cleaving protease ADAMTS thirteen. Ultralarge von Willebrand multimers accumulate, bind platelets, and form microthrombi that occlude small vessels and shear red cells, producing schistocytes and lactate dehydrogenase elevation.

The classic pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurologic symptoms, fever, and renal injury is rarely complete. Diagnosis is supported by a PLASMIC score above five, microangiopathic features on smear, and ADAMTS thirteen activity below ten percent.

Initial therapy combines daily therapeutic plasma exchange and corticosteroids. Caplacizumab, an anti von Willebrand factor nanobody, accelerates platelet recovery, and rituximab is added in refractory or relapsing disease. Untreated mortality exceeds ninety percent, but modern combined therapy reduces mortality to under twenty percent.

Symptoms

Fluctuating neurologic deficits and headache
Fatigue and pallor from hemolysis
Petechiae and purpura from thrombocytopenia
Fever and chills
Kidney injury with rising creatinine

Risk Factors

Female sex in young to middle adulthood
Autoimmune disease such as lupus
Ticlopidine clopidogrel or quinine exposure
Pregnancy or postpartum period
Human immunodeficiency virus infection

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When microangiopathic hemolysis is suspected
  • When platelet count drops abruptly
  • When neurologic symptoms appear
  • When fever and kidney injury accompany cytopenia
  • When PLASMIC score exceeds five

Treatment Methods

01
Urgent ADAMTS thirteen activity testing
02
Daily therapeutic plasma exchange
03
High dose corticosteroids
04
Caplacizumab for accelerated recovery
05
Rituximab for refractory or relapsing disease
06
Avoidance of platelet transfusion unless life threatening bleeding
07
Long term hematology followup for relapse

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Hematoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.