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General Medicine

Thrombotic Thrombocytopenic Purpura

Samata Pathireddy, MD, Narothama Reddy Aeddula, MD, FACP, FASN and Krishna M. Baradhi, MD Reviewed 06/2020
 


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.
2
George JN. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med. &#...

ADDITIONAL READING

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

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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FIGURE 20-28. Thrombotic thrombocytopenic purpura. Microthrombi are present in the brain (A) and heart (B) of a patient who died of thrombotic thrombocytopenic purpura.
FIGURE 20-28. Thrombotic thrombocytopenic purpura. Microthrombi are present in the brain (A) and heart (B) of a patient who died of thrombo...
FIGURE 20-29. Microangiopathic hemolytic anemia. Numerous schistocytes (arrows) are present in a patient with thrombotic thrombocytopenic purpura.
FIGURE 20-29. Microangiopathic hemolytic anemia. Numerous schistocytes (arrows) are present in a patient with thrombotic thrombocytopenic p...
Buerger disease. (A) Section of the upper extremity shows an organized arterial thrombus, which has occluded the lumen. Some inflammatory cells are evident in the adventitial fat. In this instance, the vein (arrow) and the adjacent nerve (arrowhead) show foci of chronic inflammation.
Buerger disease. (A) Section of the upper extremity shows an organized arterial thrombus, which has occluded the lumen. Some inflammatory c...
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