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Start free with EleplanImmune-mediated thrombotic thrombocytopenic purpura
ORPHA:93585Clinical subtype
Also called Acquired TTP · Acquired thrombotic thrombocytopenic purpura · Autoimmune thrombotic thrombocytopenic purpura · Thrombotic thrombocytopenic purpura due to anti-ADAMTS-13 antibodies · aTTP · iTTP
What it is
A rare, non-hereditary thrombotic thrombocytopenic purpura (TTP), characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and single or multiple organ failure of variable severity.
Key facts
- Prevalence
- 1-9 / 1 000 000 (annual incidence, Europe)
- Age of onset
- All ages
- Inheritance
- Multigenic/multifactorial
- Classified as
- Clinical subtype
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
4Common30–79%
25- Abdominal pain
- Arthralgia
- Coma
- Confusion
- Diarrhea
- Dyspnea
- Fatigue
- Fever
- Headache
- Hematuria
- Hyperbilirubinemia
- Increased circulating lactate dehydrogenase concentration
- Increased circulating troponin I concentration
- Intestinal ischemia
- Low back pain
- Muscle weakness
- Myalgia
- Nausea
- Proteinuria
- Reduced haptoglobin level
- Reticulocytosis
- Schistocytosis
- Seizure
- Stroke
- Unconjugated hyperbilirubinemia
Sometimes5–29%
12- Abnormal EKG
- Acute kidney injury
- Aphasia
- Arrhythmia
- Cardiogenic shock
- Chest pain
- Congestive heart failure
- Diplopia
and 4 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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