📂 Diseases
thrombotic thrombocytopenic purpura — Definition
Trombotik trombositopenik purpura / thrombotic thrombocytopenic purpura
thrombotic thrombocytopeniaopenic purpura (TTP) is a hematological disease, characterized by widespread clot formation in small blood vessels, rare and threatens life. This situation occurs with abnormal accumulation of Willebrand factor, as a result of the deficiency or dysfunction of ADAMTS13 enzyme. The clinically thrombocytopenia manifests itself with microangiopathic hemolytic anemia, neurological findings, kidney failure and fever. Emergency plasma change and immunosuppressive treatment is a disease that requires.
Disease Definition
🔬 Disease Definition
Trombotik trombositopenik purpura (TTP), mikroanjiyopatik hemolitik anemi, trombositopeni, nörolojik bulgular, böbrek yetmezliği ve ateş ile karakterize, hayatı tehdit eden bir hematolojik acil durumdur. ADAMTS13 enzim eksikliği veya inhibisyonu sonucu gelişir.
🧬 Causes & Risk Factors
Konjenital (Upshaw-Schulman sendromu) veya edinsel (otoantikorlar) ADAMTS13 eksikliği. Tetikleyiciler arasında enfeksiyonlar, gebelik, ilaçlar (kinin, tiklopidin), otoimmün hastalıklar ve maligniteler bulunur.
🩺 Symptoms & Signs
Petesi ve purpura, sarılık, halsizlik, ateş, nörolojik semptomlar (baş ağrısı, konfüzyon, nöbet, fokal defisit), böbrek yetmezliği (oligüri, hematüri), kardiyak iskemi, karın ağrısı.
📋 Diagnostic Methods
Klinik bulgular, periferik yaymada şistositler, trombositopeni (<30.000/μL), LDH yüksekliği, indirekt bilirubin artışı, ADAMTS13 aktivitesi <%10 ve/veya inhibitör varlığı.
💊 Treatment Options
Acil plazma değişimi (PEX) günlük, yüksek doz kortikosteroid, rituksimab (refrakter/relaps), kaplizumab (anti-vWF nanobody), destek tedavisi (trombosit transfüzyonu kontrendike).
⚠️ Complications
Böbrek yetmezliği, serebrovasküler olay, miyokard enfarktüsü, pulmoner ödem, relaps, tedaviye bağlı komplikasyonlar (kateter enfeksiyonu, alerjik reaksiyonlar).
Frequently Asked Questions
❓ What is Trombotik trombositopenik purpura?
Trombotik trombositopenik purpura; thrombotic thrombocytopeniaopenic purpura (TTP) is a hematological disease, characterized by widespread clot formation in small blood vessels, rare and threatens life. This situation occurs with abnormal accumulation of Willebrand factor, as a result of the deficiency or dysfunction of ADAMTS13 enzyme. The clinically thrombocytopenia manifests itself with microangiopathic hemolytic anemia, neurological findings, kidney failure and fever. Emergency plasma change and immunosuppressive treatment is a disease that requires.
❓ What is thrombotic thrombocytopenic purpura in Turkish?
The Turkish equivalent of "thrombotic thrombocytopenic purpura" is Trombotik trombositopenik purpura.
❓ Which medical field is Trombotik trombositopenik purpura related to?
This term belongs to the Diseases category.