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Thrombotic Thrombocytopenic Purpura

Theresa A. Townley, MPH, MD and Rutendo JOKOMO-Nyakabau, MD, Doctor of Medicine Reviewed 06/2022
 


BASICS

DESCRIPTION

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

DIAGNOSIS

  • Most common symptoms are nonspecific: nausea, vomiting, weakness, abdominal pain, fatigue, fever.

  • Related to thrombocytopenia - Easy bruising, purpura, or petechiae, Epistaxis, menorrhagia, b...

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

Maintenance therapy is not required. After PEX is discontinued, blood counts should be monitored for months. If results remain normal, testing interval can be len...

REFERENCES

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

ADDITIONAL READING

Joly BS , Coppo P, Veyradier A. An update on pathogenesis and diagnosis of thrombotic thrombocytopenic purpura. Expert Rev. Hematol. 2019;12(6):383–395. ...

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 historical p...

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