Thelansis

  Thrombotic thrombocytopenic purpura (TTP) is a form of microangiopathic hemolytic anemia characterized by the classic pentad of symptoms: fever, thrombocytopenia, hemolytic anemia, renal dysfunction, and neurologic dysfunction. TTP arises from a deficiency or absence of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13), either congenital or acquired. Reduced levels of ADAMTS13 lead to the formation of microthrombi, resulting in ischemic damage to various organs. ADAMTS13 cannot effectively inhibit the large multimer von Willebrand factor (VWF), crucial for preventing spontaneous coagulation. Differential diagnoses encompass other thrombotic microangiopathies (TMAs), such as hemolytic uremic syndrome and TMA, secondary to factors like transplantation, chemotherapy, drugs, HIV/AIDS, and HELLP syndrome. Other conditions include catastrophic antiphospholipid syndrome, severe sepsis, vitamin B12 deficiency, malignant hypertension, and dissem...