Hemophilia, Thrombocytopenia, Sickle Cell Flashcards
hemostasis
- initiating clot formation to stop bleeding
- involves an interaction among the blood vessel wall, platelets and plasma coagulation proteins and a number signaling molecules
Von Willebrand’s Disease
- caused by a lack of or dysfunction of vWF
- can causes excessive bleeding because they do not have the factor to enhance adhesiveness of platelets
- most common inherited bleeding disorder
vWF
von willebrand factor
- released from platelets following vascular injury
hematoma
collection of blood outside of blood vessels
hemarthrosis
blood moving into the joints
hemophilia
bleeding disorder inherited as a sex-linked autosomal recessive trait (X chromosome)
what is hemophilia caused by
lack/deficiency of/in a clotting factor
how is hemophilia classified
according to the percentage of clotting factor present in plasma
when do you see hemophilia with newborns
occur with heel sticks, immunizations, blood draws, circumcision
when do you see hemophilia with adults
- bruising
- bleeding from the mouth or frenulum
- intracranial bleeding
- hematomas of the head
- hemarthrosis with ambulation
when do you see hemophilia in women
- excessive bleeding during menstrual cycle
- abnormal bleeding from bruising
- dental extractions
- abortion/miscarriage
- complication with pregnancy
- nosebleeds
- minor trauma
how would hemophilia affect joints?
- hemarthrosis, most common clinical manifestations
- causes the joint to become distended, causing swelling, pain, warmth and stiffness
how can weight bearing affect the joints with hemophilia?
may further damage the synovium –> hypertrophic synovium which leads to further bleeding, swelling, narrowing of the joint space and joint pain
what would you see with muscle clinical manifestations and hemophilia
- muscle hemorrhages/space occupying
- often flexor muscles
- pain and limited ROM
- can compress neurovascular bundle
hemophilia A
- lack of the clotting factor VIII
- 80% of all cases
