Bleeding and Clotting - Hubbard Flashcards
platelet adhesion is dependent on glycoproteins on platelet surface and is mediated by vonWillebrand factor
- activated platelets have storage granules and secrete factors (ADP, serotonin) which recruit other platelets
- end result is the formation of a platelet plug which blocks egress of blood from the site of vascular injury
primary hemostasis
involves the serum coagulation factors which ultimately catalyze the devlpment of fibrin latticework which braces and supports the platelet plug
- serum coagulation factors also function to recruit platelets and amplify both primary and secondary hemostasis
secondary hemostasis
what ROS are important to ask in a pt with bleeding disorder?
- bleeding
- bruising
- abnormal clotting
- recurrent DVT
- hx of PE/stroke
- fatigue
- fever
- enlargement of spleen/LN/liver
- weight loss
- GO bleed
- GU bleed -> beware beeturia!
what should always be considered a cause of petechiae/thrombocytopenia?
medications:
- abx (cephalosporin)
- H2 blockers
- heparin
- digoxin
complication of medical, surgical, obstetrical situations
- intrinsic and extrinsic coagulation system are activated with resulting local and general escape of thrombin into the circulatory system, resulting in initial thrombosis
- a platelets and clotting factors are depleted, bleeding ensues -> major factor of dz
disseminated intravascular coagulation (DIC)
what is the tx for DIC?
- correction of underlying dz: sepsis, bowel obstruction
- heparin (only if thrombosis occurs)
- supportive care
generalized disorder of microcirculation characterized by thrombocytopenic purpura, microangiopathic hemolytic anemia (schistocytes), fluctuating neurological signs, febrility
thrombotic thrombocytopenic purpura (TTP)
- hyaline thrombi which occlude the capillaries of virtually every organ in the body
MAHA + thrombocytepenia (<50k) + fever + neurologic symptoms
TTP
MAHA + thrombocytopenia (<50k) + fever + neurologic symptoms + RENAL FAILURE
hemolytic uremic syndrome (HUS)
what are the two major forms of TTP?
- hereditary: ADAMTS13 mutation
- acquired: autoAb against ADAMTS13 (linked to infection, malignancy, immune disorders)
No ADAMTS13 = too much clumping
what is the tx for TTP?
- treat the underlying cause
- plasmapharesis
- nearly 100% mortality if not treated
very large group of related disease that have decreased platelet adhesion as their hallmark
vonWillebrand disease
- pathologic lesion is decreased or absent vWF
what is the tx for vonWillebrand disease?
- cryoprecipitate (replaces vWF)
- DDAVP (causes release of vWF from endothelium
defects in platelet function may be seen in what 3 diseases?
- uremia: impaired platelet adhesion
- dysproteinemias: interference with platelet membrane function
- autoimmune disorders: multiple abnormalities
the only endothelial syndrome associated with hemostatic complications
- caused by thinning of vessel walls with telangiectatic formations, AV malformations, and aneurysmal dilations throughout the body
- AD inheritance
- telangiectasias gradually appear throughout life
- epistaxis MC sx
hereditary hemorrhagic telangiectasia
- aka Osler-Weber-Rendu syndrome
hereditary hemorrhagic telangiectasia is caused by a defect in what gene?
endoglin (CD105) on xsome 9
- membrane glycoprotein strongly expressed on endothelial cells
what is the tx for hereditary hemorrhagic telangiectasia?
surgery and laser photoablation of telangiectasias of some value
- usually benign clinical course, recurrent bleeds frequent, but death from exsanguination is rare
recurrent LE thrombophlebitis and DVT, venous insufficiency, chronic leg ulcers
- significantly increased risk DVT in pregnant women (due in part to pregnancy-induces hypercoagulability)
AT-III deficiency
- diagnosed by decreased levels AT-III in serum (<50%)
what is the tx for AT-III def?
- prophylactic w/ anticoagulants
- pts w/ DVT should receive heparin, HIGH doses
- AT-III replacement therapy is available for pts w/DVT