This document summarizes von Willebrand's disease, the most common inherited bleeding disorder. It is characterized by defective platelet adhesion and aggregation due to reduced or dysfunctional von Willebrand factor. The disorder was first described in 1926 and is caused by mutations in the von Willebrand factor gene. It is classified into types 1, 2, and 3 based on von Willebrand factor levels and functionality. The disease primarily causes mucocutaneous bleeding and bleeding after procedures or trauma. Treatment aims to replace von Willebrand factor levels through desmopressin or factor concentrates.
By Srivardhan Vanka, Intern - Batch of 2014, J.I.P.M.E.R.
References from UpToDate and Wintrobe's Hematology 13th Edition and various studies done till December 2019. Quoted when appropriate.
Von Willebrand Disease is the most common hereditary bleeding disorder; roughly 1 in every 100 people suffers from the disease. People who suffer from VWD have blood that does not clot properly.
Normally when a person is injured and starts to bleed, the von Willebrand factor in the blood attaches to small blood cells called platelets. This helps the platelets stick together to form a clot at the site of the injury and stop bleeding. When a person has VWD, the clot might take longer to form or not form the way it should and bleeding might take longer to stop. This can lead to heavy, hard-to-stop bleeding. Although rare, the bleeding can be severe enough to damage joints or internal organs, or even be life-threatening.
http://www.nlm.nih.gov/medlineplus/plateletdisorders.html#cat1
http://www.cdc.gov/ncbddd/vwd/facts.html
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
a powerpoint on hemophilia i needed to make.
**if you want to join an online class if you are interested in history, science, math, english, and other things, join my class!!**
By Srivardhan Vanka, Intern - Batch of 2014, J.I.P.M.E.R.
References from UpToDate and Wintrobe's Hematology 13th Edition and various studies done till December 2019. Quoted when appropriate.
Von Willebrand Disease is the most common hereditary bleeding disorder; roughly 1 in every 100 people suffers from the disease. People who suffer from VWD have blood that does not clot properly.
Normally when a person is injured and starts to bleed, the von Willebrand factor in the blood attaches to small blood cells called platelets. This helps the platelets stick together to form a clot at the site of the injury and stop bleeding. When a person has VWD, the clot might take longer to form or not form the way it should and bleeding might take longer to stop. This can lead to heavy, hard-to-stop bleeding. Although rare, the bleeding can be severe enough to damage joints or internal organs, or even be life-threatening.
http://www.nlm.nih.gov/medlineplus/plateletdisorders.html#cat1
http://www.cdc.gov/ncbddd/vwd/facts.html
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
a powerpoint on hemophilia i needed to make.
**if you want to join an online class if you are interested in history, science, math, english, and other things, join my class!!**
Factor v deficiency is rare
first described in a Norwegian patient in 1943, Identified by Dr. Paul Owren .
Fewer than 200 cases of congenital factor V deficiency have been reported worldwide since 1943.
inheritance of factor V deficiency is autosomal recessive.
usually only needed for severe bleeds or before surgery.
there is no concentrate containing only factor V.
fresh plasma or (FFP) infusions are used to correct the deficiency temporarily and should be given daily during a bleeding episode.
My son had Wiskott Aldrich Syndrome (WAS). He had a bone marrow transplant in August 2006. His WAS is healed. This presentation was designed by some grad students. Some of the content is from my blog and it pictures my son, David. http://www.davidmcnally.blogspot.com
Inherited bleeding disorders are usually associated with boys and men. But with medical advances, more and more women have been diagnosed with inherited bleeding disorders such as Hemophilia, von Willebrand Disease, Platelet Function Disorders and factor deficiency disorders. This presentation shares about bleeding disorders affecting women. The first part is a report from the WFH World Congress 2014 held in Melbourne by the presenter, My Girls Blood Ambassador from the Philippines, Andrea Echavez. The second part is about inherited bleeding disorders affecting women.
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