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Thrombotic Thrombocytopenic Purpura

Samata Pathireddy, MD, Narothama Reddy Aeddula, MD, FACP, FASN and Krishna M. Baradhi, MD Reviewed 06/2019
 


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, bleedi...

TREATMENT

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

  • Complete response to treatment is defined by a platelet count >150 × 109/L for 2 consecutive days, together with ...

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]
2
George JN. Clinical practice. Thrombotic thrombocyto...

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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