DISEASE SCANNER

Global Incurable Diseases Tracker

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Hematologic Disorder

Thrombotic Thrombocytopenic Purpura (TTP)

HIGH SEVERITY

Rare, life-threatening blood disorder causing blood clots in small blood vessels throughout the body. Characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia. Caused by ADAMTS13 deficiency or inhibitors.

Global Affected

10.0K

Countries

15

Symptoms

Purpura and bruising
Fatigue
Fever
Neurological symptoms
Renal dysfunction
Jaundice
Chest pain
Shortness of breath

Treatment Options

Plasma exchange
Fresh frozen plasma infusion
Corticosteroids
Rituximab
Caplacizumab
Splenectomy (refractory)
Immunosuppressants

Risk Factors

1Autoimmune disorders
2Pregnancy
3Infections
4Certain medications
5Genetic mutations (Upshaw-Schulman syndrome)
6Stem cell transplant

Diagnostic Methods

  • 1Complete blood count
  • 2Peripheral blood smear (schistocytes)
  • 3ADAMTS13 activity and inhibitor levels
  • 4LDH level
  • 5Reticulocyte count
  • 6Coombs test

Prognosis

Without treatment, mortality exceeds 90%. With plasma exchange, survival improves to 80-90%. Caplacizumab further reduces mortality. Relapse occurs in 30-50% of autoimmune TTP. Long-term follow-up essential. Neurological and renal complications can persist. Early diagnosis and treatment critical.

Prevention

  • No primary prevention
  • Regular ADAMTS13 monitoring (remission)
  • Genetic counseling (hereditary form)
  • Avoiding triggers (known precipitants)

Research Status

Plasma exchange (plasmapheresis) is first-line treatment. Caplacizumab (anti-vWF nanobody) reduces mortality. Corticosteroids and rituximab for autoimmune TTP. Splenectomy for refractory cases. Recombinant ADAMTS13 in development.

Affected Countries

United StatesUnited KingdomCanadaAustraliaGermanyFranceItalySpainJapanBrazilMexicoNigeriaSouth AfricaIndiaChina

Sources

  • https://www.hematology.org
  • https://www.ncbi.nlm.nih.gov/books/NBK6614
  • https://www.mayoclinic.org

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.