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Von Willebrand Disease (VWD) | The Most COMPREHENSIVE Explanation!

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Medicosis Perfectionalis

VonWillebrand factor (vWF) comes from the meagakaryocytes (bone marrow) as well as the WeibelPalade bodies (endothelial cells). Von Willebrand factor (vWF) helps the platelets adhere to the sub endothelial collagen via GP1b receptor on the platelet.

Von Willebrand disease (vWD) is the most common inherited bleeding disorder worldwide.

Primary hemostasis involves forming a platelet plug (thrombocytes) whereas secondary hemostasis involves making a fibrin thrombus (coagulation factors).

Ionized calcium helps blood coagulate.

We test for primary hemostasis via platelet count, bleeding time, and platelet aggregometry.
We test secondary Hemostasis via prothrombin time (PT), activated partial thromboplastin time (aPTT), and the old coagulation time (or clotting time, or thrombin time).

Von Willebrand Dusease (vWD) affects primary as well as secondary Hemostasis.

Hemolytic Uremic Syndrome (HUS), atypical Hemolytic Uremic Syndrome (aHUS), and Thrombotic Thrombocytopenic Syndrome (TTP).

Atypical hemolytic uremic syndrome (aHUS) is similar to thrombotic thrombocytopenic Purpura (TTP).

Typical Hemolytic Uremic Syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.

Thrombotic thrombocytopenic purpura (TTP) is a pentad of microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure, fever, and neurological abnormalities.

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BernardSoulier Syndrome (BSS) is an autosomal recessive (AR) disorder characterized by a defective GP1b platelet receptor leading to a problematic platelet adhesion process (primary hemostasis).

Glanzmann Thrombasthenia (GT) is an autosomal recessive defect in the GPIIb/IIIa (GP2b/3a) receptor on the platelet which leads to defective platelet aggregation.

Ristocetin Cofactor Assay (RIPA) is usually abnormal in cases of BernardSoulier Syndrome (BSS).
Bleeding time is prolonged, platelets are big in size, but few in number (Macrothrombocytopenia).

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Platelet count is one of the lab tests used by a doctor or a hematologist to assess whether your platelet number is normal, low (thrombocytopenia) or high (thrombocytosis).

Bleeding time (BT) is another test for platelet function.

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