vWF is a large glycoprotein present in the plasma whose functions include binding factor VIII, and activating and binding platelets in response to endothelial injury. It is produced in the endothelium as ultra-large multimers that are inactivated when cleaved by...
Primary Congenital (5%) - typically present in late infancy or childhoodAcquired idiopathic (autoimmune) Secondary(To a known Trigger) Infection:HIVCMVHBV / HCVPregnancyBone marrow transplantDrugs:COCP and hormonal treatmentsQuinine Interferon and...
TTP is said to present with a classic pentad of features: However, not all features are required to be present to make a diagnosis Additional features are also commonly seen in TTP Patients report feeling unwell for several weeks before presenting: Usually, experience...
Features of MAHA: Anaemia on FBC Blood film: low platelets, schistocytes, polychromasia Increased reticulocytes, bilirubin and LDH Negative DAT Coomb’s test Marked thrombocytopenia Decreased ADAMTS13 activity Anti-ADAMTS13 IgG may be...
Severe disease is complicated by organ failure: Neurological Renal Cardiovascular Other organs SeizuresParalysisIschaemic ChangesCerebral BleedingComa Renal Failure Acute myocardial infarctionArrhythmiasHeart failureCardiogenic shock PancreatitisGI BleedingMesenteric...
