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Thrombotic Thrombocytopenic Purpura

Erin Connor, MD, Keith Petersen, DO and Stephanie Rosen, MD Reviewed 06/2018
 


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, blee...

TREATMENT

ALERT

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

  • In the absence of another apparent cause, the dyad of MAHA and thrombocytopenia is sufficient to begin treatment for...

ONGOING CARE

FOLLOW UP RECOMMENDATIONS

  • No maintenance therapy is required. After PEX is discontinued, blood counts should be monitored over a few months. If testing results remain normal, testing inter...

REFERENCES

1
Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood.  2008;112(1):11–18. [View Abstract on OvidMedline]
2
Reese JA, Muthurajah DS, Kremer Hovinga...

ADDITIONAL READING

George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med.  2014;371(7):654–666. [View Abstract on OvidMedline] 

CODES

ICD10

  • M31.1 Thrombotic microangiopathy

  • D69.42 Congenital and hereditary thrombocytopenia purpura

  • D69.3 Immune thrombocytopenic purpura

ICD9

  • 446.6 Thrombotic microangiopathy

  • 287.33 Congenital and hered...

CLINICAL PEARLS

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

  • The histo...

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