ORPHA:93585
Immune-mediated thrombotic thrombocytopenic purpura
Synonyms: Acquired TTP · Acquired thrombotic thrombocytopenic purpura · Autoimmune thrombotic thrombocytopenic purpura · Thrombotic thrombocytopenic purpura due to anti-ADAMTS-13 antibodies · aTTP · iTTP
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- All ages
- Status
- active
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.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immune-mediated thrombotic thrombocytopenic purpura | Acquired TTP · Acquired thrombotic thrombocytopenic purpura · Autoimmune thrombotic thrombocytopenic purpura · Thrombotic thrombocytopenic purpura due to anti-ADAMTS-13 antibodies · aTTP · iTTP |
| fr | Purpura thrombotique thrombocytopénique d'origine immunitaire | PTT acquis · Purpura thrombotique thrombocytopénique acquis · Purpura thrombotique thrombocytopénique associé aux anticorps anti-ADAMTS-13 · Purpura thrombotique thrombocytopénique d'origine auto-immune · TTP acquis |
| de | Thrombotisch-thrombozytopenische Purpura, immun-vermittelte | Autoimmune thrombotisch-thrombozytopenische Purpura · Erworbene TTP · aTTP · iTTP |
| es | Púrpura trombótica trombocitopénica inmunomediada | PTT adquirida · Púrpura trombocitopénica trombótica autoinmune · Púrpura trombocitopénica trombótica por anticuerpos anti-ADAMTS-13 |
| it | Porpora trombotica trombocitopenica immuno-mediata | Porpora trombotica trombocitopenica acquisita da anticorpi anti-ADAMTS 13 · Porpora trombotica trombocitopenica autoimmune · TTP acquisita |
| nl | Immuungemedieerde trombotische trombocytopenische purpura | Auto-immune trombotische trombocytopenische purpura · Trombotische trombocytopenische purpura door anti-ADAMTS-13-antilichamen · Verworven TTP · Verworven trombotische trombocytopenische purpura · aTTP · iTTP |
| pt | Púrpura trombocitopénica trombótica imunomediada | Púrpura trombocitopénica trombótica adquirida por anticorpos anti-ADAMTS 13 |
| pl | Nabyta plamica zakrzepowa małopłytkowa | Autoimmunologiczna plamica zakrzepowa małopłytkowa · Nabyta TTP |
| cs | Získaná trombotická trombocytopenická purpura | Získaná TTP · Autoimunitní trombotická trombocytopenická purpura |
| uk | Імуноопосередкована тромботична тромбоцитопенічна пурпура | Набута тромботична тромбоцитопенічна пурпура · Набутий ТТП · aTTП · Аутоімунна тромботична тромбоцитопенічна пурпура · iTTП · Тромботична тромбоцитопенічна пурпура, спричинена анти-АДАМТС-13 антитілами |
| zh | 获得性血栓性血小板减少性紫癜 | 抗ADAMTS抗体所致血栓性血小板减少性紫癜 |
| tr | Edinsel trombotik trombositopenik purpura | Edinsel TTP · Otoimmün trombotik trombositopenik purpura · Anti-ADAMTS-13 antikorlarına bağlı trombotik trombositopenik purpura |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M31.1 | NTBT | attributed | validated |
| ICD-11 | 3B64.14 | NTBT | index | validated |
| MONDO | 0019740 | E | validated | |
| MeSH | C536901 | E | validated | |
| UMLS | C2584778 | E | validated | |
| GARD | 4607 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:54057Thrombotic thrombocytopenic purpura
rare renal diseases; rare hematological diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | — | Europe | Class only | Validated | ORPHANET |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
41 annotations · validated 2024-02-28| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:6000462 | Anti-ADAMTS13 antibody positivity | |
| HP:0001937 | Microangiopathic hemolytic anemia | |
| HP:0000979 | Purpura | |
| HP:0001873 | Thrombocytopenia | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002829 | Arthralgia | |
| HP:0001259 | Coma | |
| HP:0001289 | Confusion | |
| HP:0002014 | Diarrhea | |
| HP:0002094 | Dyspnea | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0002315 | Headache | |
| HP:0000790 | Hematuria | |
| HP:0002904 | Hyperbilirubinemia | |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0410173 | Increased circulating troponin I concentration | |
| HP:0033404 | Intestinal ischemia | |
| HP:0003419 | Low back pain | |
| HP:0001324 | Muscle weakness | |
| HP:0003326 | Myalgia | |
| HP:0002018 | Nausea | |
| HP:0000093 | Proteinuria | |
| HP:0020181 | Reduced haptoglobin level | |
| HP:0001923 | Reticulocytosis | |
| HP:0001981 | Schistocytosis | |
| HP:0001250 | Seizure | |
| HP:0001297 | Stroke | |
| HP:0008282 | Unconjugated hyperbilirubinemia | |
| Occasional (29-5%) | ||
| HP:0003115 | Abnormal EKG | |
| HP:0001919 | Acute kidney injury | |
| HP:0002381 | Aphasia | |
| HP:0011675 | Arrhythmia | |
| HP:0030149 | Cardiogenic shock | |
| HP:0100749 | Chest pain | |
| HP:0001635 | Congestive heart failure | |
| HP:0000651 | Diplopia | |
| HP:0003259 | Elevated circulating creatinine concentration | |
| HP:0003138 | Increased blood urea nitrogen | |
| HP:0001658 | Myocardial infarction | |
| HP:0001645 | Sudden cardiac death | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 33540569[PMID]_30531363[PMID]_28416507[PMID]_28382967[PMID]_31107120[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/93585
- This record as JSON: /data/d/93585.json