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

Thrombotic thrombocytopenic purpura - Skin

See also in: Overview
Print Images (4)
Contributors: Art Papier MD, William Van Stoecker MD
Other Resources UpToDate PubMed

Synopsis

Thrombotic thrombocytopenic purpura (TTP) is a severe, life-threatening disease characterized by the pentad of thrombocytopenia, neurologic abnormalities, fever, microangiopathic hemolytic anemia, and renal dysfunction. Microvascular occlusions with platelet thrombi occur throughout the body, leading to the clinical manifestations. The pathogenesis appears to be related to a deficiency in a von Willebrand factor-cleaving protease termed ADAMTS 13. The cause may be idiopathic (autoimmune), secondary to an underlying process, or genetic (Upshaw-Schulman syndrome).

The symptoms of TTP are nonspecific and may include fever, fatigue, malaise, arthralgias, altered mental status, seizures, hemiplegia, abdominal pain, nausea, and vomiting. TTP is slightly more common in women, and the average age of onset is approximately 35-40 years.

Thrombocytopenic purpura may also be associated with malignancy, pregnancy, HIV infection, certain medications, and the post-transplantation state. In 2012, multiple cases of TTP-like illness were associated with dissolving and injecting tablets intended for oral administration of a reformulated extended-release form of oxymorphone (Opana ER).

With prompt diagnosis and plasma exchange, the survival rate of TTP has increased from 5%-10% to 80%-90%. When complications occur, they include stroke, arrhythmia, myocardial infarction, hemorrhage, and renal failure, although many survivors suffer no permanent sequelae.

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

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 Updated: 06/04/2013
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Emergency: requires immediate attention
Thrombotic thrombocytopenic purpura - Skin
See also in: Overview
Print 4 Images
View all Images (4)
(with subscription)
Thrombotic thrombocytopenic purpura (General Manifestations) : Altered mental state, Fever, Jaundice, Hemolytic anemia, Malaise, Microscopic hematuria, Arthralgia, Pallor, PLT decreased, Legs
Clinical image of Thrombotic thrombocytopenic purpura
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