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Emergency: requires immediate attention
Thrombotic thrombocytopenic purpura
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Emergency: requires immediate attention

Thrombotic thrombocytopenic purpura

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Contributors: Connie R. Shi, Paritosh Prasad MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Thrombotic thrombocytopenic purpura (TTP) is a severe, life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction.

TTP is a subtype of the thrombotic microangiopathy (TMA) syndromes. Other subtypes of TMA include Shiga toxin-mediated TMA (Shiga toxin hemolytic-uremic syndrome), drug-mediated TMA, and complement-mediated TMA, among others. Accurate diagnosis of TTP and prompt initiation of treatment are essential to prevent morbidity and mortality.

The cause may be acquired or, in rare cases, hereditary (Upshaw-Schulman syndrome). In acquired TTP, autoantibodies form against ADAMTS13, which normally cleaves von Willebrand factor (vWF). As a result, vWF multimers accumulate, causing platelet aggregation and microvascular occlusion. Upshaw-Schulman syndrome results from genetic mutations in the ADAMTS13 gene.

Classically, TTP was thought to be associated with a "pentad" of clinical and laboratory findings including microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever, and renal dysfunction. However, the latter 3 findings are not reliably present in TTP, and consequently the presence of microangiopathic hemolytic anemia and thrombocytopenia are sufficient for a diagnosis of TTP.

Clinical features of TTP are varied and may include gastrointestinal symptoms (abdominal pain, nausea, vomiting), fatigue, malaise, neurologic abnormalities (altered mental status, confusion, headache), and purpuric rash.

TTP is more common in women and in Americans of African descent. Hereditary TTP usually presents before age 5. Acquired TTP typically presents between the ages of 30 and 50.

Codes

ICD10CM:
M31.1 – Thrombotic microangiopathy

SNOMEDCT:
78129009 – Thrombotic thrombocytopenic purpura

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Thrombotic microangiopathy syndromes (microangiopathic hemolytic anemia, thrombocytopenia):
  • Shiga toxin-mediated TMA (hemolytic-uremic syndrome) – diarrheal illness as prodrome; severe renal dysfunction
  • Drug-mediated TMA (quinine, quetiapine, gemcitabine, and calcineurin inhibitors, among others)
  • Complement-mediated TMA
The following conditions could also be considered in the differential of microangiopathic hemolytic anemia and thrombocytopenia:
During pregnancy:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 03/02/2017
Last Updated: 06/21/2017
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Emergency: requires immediate attention
Thrombotic thrombocytopenic purpura
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View all Images (3)
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Thrombotic thrombocytopenic purpura : Abdominal pain, Altered mental state, Fatigue, Fever, Headache, Jaundice, Nausea
Clinical image of Thrombotic thrombocytopenic purpura
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