Classifications
- Thrombotic microangiopathy (TMA):
- TTP (see below)
- Hemolytic uremic syndrome:
- Infection-associated HUS (IA-HUS)
- Complement-mediated HUS (CM-HUS)
- Other:
- Connective tissue disease
- Transplantation
- Disseminated intravascular coagulation (DIC)
- Pregnancy-associated TMAs:
- HELLP syndrome
- Pre-eclampsia
- Drugs
- Pancreatitis
- Malignancy
- Streptococcus pneumonia
- MAHA:
- In presence of platelet or fibrin clots:
- TTP
- HUS
- Disseminated malignancy
- Malignant hypertension
- Severe preeclampsia
- Cavernous hemangioma
- Disseminated intravascular coagulation (DIC)
- In absence of platelet or fibrin clots:
- Prosthetic heart valves
- Ventricular assisted devices
- Extracorporeal membrane oxygenation
- In presence of platelet or fibrin clots:
- Thrombotic thrombocytopenic purpura (TTP):
- Congenital TTP (cTTP) (Upshaw–Schulman syndrome)
- Immune mediated TTP (iTTP) (also called autoimmune TTP or acquired TTP):
- Primary iTTP:
- No obvious underlying precipitating cause/disease.
- ADAMTS-13 activity of< 10% with the presence of ADAMTS-13 autoantibodies.
- Secondary iTTP:
- Precipitating cause (underlying disorder or trigger) can be identified, including:
- Connective tissue disease:
- SLE
- Sjogren’s syndrome
- Rheumatoid arthritis
- HIV infection
- Cytomegalovirus infection
- Pregnancy
- Drugs:
- Ticlodipine
- Quinine
- Simvastatin
- Trimethoprim
- Pegylated interferon
- Connective tissue disease:
- ADAMTS-13 activity of< 10% with the presence of ADAMTS-13 autoantibodies.
- Precipitating cause (underlying disorder or trigger) can be identified, including:
- Primary iTTP: