Thrombotic thrombocytopenic purpura | |
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Other names | Moschcowitz syndrome,[1] idiopathic thrombotic thrombocytopenic purpura[2] |
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Spontaneous bruising in a woman with critically low platelets | |
Specialty | Hematology |
Symptoms | Large bruises, fever, weakness, shortness of breath, confusion, headache[3][2] |
Usual onset | Adulthood[3] |
Causes | Unknown, bacterial infections, certain medications, autoimmune diseases, pregnancy[3] |
Diagnostic method | Based on symptoms and blood tests[2] |
Differential diagnosis | Hemolytic-uremic syndrome (HUS), atypical hemolytic uremic syndrome (aHUS)[4] |
Treatment | Plasma exchange, immunosuppressants[1] |
Prognosis | < 20% risk of death[1] |
Frequency | 1 in 100,000 people[3] |
Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body.[2] This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, heart, and brain dysfunction.[1] Symptoms may include large bruises, fever, weakness, shortness of breath, confusion, and headache.[2][3] Repeated episodes may occur.[3]
In about half of cases a trigger is identified, while in the remainder the cause remains unknown.[3] Known triggers include bacterial infections, certain medications, autoimmune diseases such as lupus, and pregnancy.[3] The underlying mechanism typically involves antibodies inhibiting the enzyme ADAMTS13.[1] This results in decreased break down of large multimers of von Willebrand factor (vWF) into smaller units.[1] Less commonly TTP is inherited, known as Upshaw–Schulman syndrome, such that ADAMTS13 dysfunction is present from birth.[5] Diagnosis is typically based on symptoms and blood tests.[2] It may be supported by measuring activity of or antibodies against ADAMTS13.[2]
With plasma exchange the risk of death has decreased from more than 90% to less than 20%.[1] Immunosuppressants, such as glucocorticoids, and rituximab may also be used.[3] Platelet transfusions are generally not recommended.[6]
About 1 per 100,000 people are affected.[3] Onset is typically in adulthood and women are more often affected.[3] About 10% of cases begin in childhood.[3] The condition was first described by Eli Moschcowitz in 1924.[3] The underlying mechanism was determined in the 1980s and 1990s.[3]
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