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Thrombotic Thrombocytopenic Purpura

Chirag N. Shah, MD, Grant Wei, FACEP, MD and Jay Patel, Doctor of Osteopath Reviewed 04/2024
 


BASICS

DESCRIPTION

  • An acute syndrome of microangiopathic hemolytic anemia (MAHA) and consumptive thrombocytopenia with deposition of hyaline thrombi in terminal arterioles and capillaries leads to isch...

DIAGNOSIS

  • Screening ADAMTS13 activity level

    • <10% defines severe deficiency, and makes TTP more likely. Levels of 10-20% suggest possible TTP especially if drawn after Plasma exchange (PEX)...

TREATMENT

ALERT
  • Prompt treatment of presumptive or confirmed TTP is necessary due to the high mortality (90%).

  • Complete response to treatment is defined by a platelet count >150 × 109/L for 2 consecu...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

After PEX is discontinued, blood counts and ADAMTS13 activity should be monitored for months to evaluate for relapse.  

PATIENT EDUCATION

  • Self-monitor for signs of ...

REFERENCES

1
Joly  BS, Coppo  P, Veyradier  A. Thrombotic thrombocytopenic purpura. Blood.  2017;129(21):2836–2846. [View Abstract on OvidMedline]
2
Micha...

ADDITIONAL READING

SEE ALSO

Chen B, Li X, Xiao D, et al. Comparison of the efficacy and safety of caplacizumab versus placebo in thrombotic thrombocytopenic purpura: a meta-analysis and s...

CODES

ICD10

  • M31.1 Thrombotic microangiopathy

  • D69.42 Congenital and hereditary thrombocytopenia purpura

  • D69.3 Immune thrombocytopenic purpura

SNOMED

  • 78129009 Thrombotic thrombocytopenic purpura (disorder)

  • 37...

CLINICAL PEARLS

  • The diagnosis of TTP is made clinically; symptoms are nonspecific: nausea; vomiting; weakness; abdominal pain; fatigue; fever; and easy bruising, purpura, or petechiae.

  • The pentad of fe...

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