Term
| Clinical features of platelet disorders: |
|
Definition
- petechiae
- ecchymsoses/hematoma
- immediate onset of bleeding after surgery or trauma
- spontaneous mucous membrane bleeding |
|
|
Term
| Clinical features of coagulation disorders: |
|
Definition
- hemarthroses
- ecchymosis/hematomas
- delayed onset of bleeding after surgery/trauma
- mucous membrane bleeding with trauma or disease |
|
|
Term
| petechiae do or do not blanch. |
|
Definition
|
|
Term
| Types of coagulation disorders: |
|
Definition
- Factor VII
- Factor VIII
- Factor IX
- Factor XI
- Thrombocytopenia:
- Idiopathic Thrombocytopenic Purpura
- Thrombotic Thrombocytopenic Purpura
- Von Willebrand's Disease |
|
|
Term
| Deficiencies of factors __, __, and __ are __ __ __ cause bleeding. These are associated with prolonged ___, while the __ is normal. |
|
Definition
- VIII, IX, and XI are sex linked recessive
- prolongedpartial thromboplastin time (PTT)
- PT Is normal |
|
|
Term
| Deficiencies of factors _, _, __, __ and __ are __ __. These are fibrinogen deficiencies that cause bleeding. You will see a prolonged __ and __. |
|
Definition
- II, V, VII, X, and XI
- autosomal recessive
- PTT and PT |
|
|
Term
| Deficiency of factor XII is __ __ and associated with bleeding and impaired wound healing. The PT and PTT will be __, but the __ __ will be abnormal. |
|
Definition
-autosomal recessive
-normal
- clot solubility |
|
|
Term
| Factor VII is a __ __ dependent coagulation factor that is synthesized in the ___. Inherited deficiency of factor VII is rare. |
|
Definition
- vitamin K dependent
- liver |
|
|
Term
| Activation of plasma ___ is the initiating event of coagulation (extrinsic). Then the TF/__ complex rapidly activates factors __, __, and __, initiating the coagulation cascade. |
|
Definition
- factor VII
- TF/FVIIa complex
- VII, IX, and X |
|
|
Term
| Most severe cases of factor VII deficiency occur during __. In infancy the most common bleeds are in the __ _ and __. |
|
Definition
- childhood (less than 6 months)
- GI tract and CNS |
|
|
Term
| Acquired factor VII deficiency is due to __ __ deficiency, __ __ __ therapy, or __ disease. Specific __ __ are required for diagnosis. |
|
Definition
- vitamin K deficiency
- vitamin K antagonist therapy
- liver diseae
- FVII assays |
|
|
Term
| Tmt of factor VII disorders; |
|
Definition
| FVII replacement therapy to stop bleeding |
|
|
Term
| Hemophilia A is __ __ recessive and is deficiency of factor __. If a father has hemophilia A and the mom is not a carrier, how will the sons and daughters turn out? |
|
Definition
- x-linked recessive
- VIII
- daughters will be carriers
- sons will be unaffected |
|
|
Term
| If mother has hemophilia trait, and the father is normal, how will sons and daughters turn out? |
|
Definition
- 50% chance that each daughter will be carrier
- 50% chance that each son will have hemophilia |
|
|
Term
| most common inherited coagulation disorder: |
|
Definition
|
|
Term
| second most common inherited coagulation disorder: |
|
Definition
Hemophilia A
99% of Hemophiliacs have Hemophilia A |
|
|
Term
Name the coagulation disorder:
- spontaneous hemarthroses
- prolonged aPTT
- normal PT
- normal bleeding time |
|
Definition
|
|
Term
|
Definition
- factor VIII concentrate
- avoid NSAIDs
- DDAVP before surgery (vasopressin analogue that causes release of factor VIII) |
|
|
Term
| Hemophilia B is deficiency of factor __ and is __ __. It can be acquired from __ __ __, __ __, and __. There will be a prolonged __. Treat with __ __ _ and __ __ ___. |
|
Definition
- factor IX
- x-linked recessive
- vitamin K deficiency
- liver disease
- coumadin
- PTT
- fresh frozen plasma
- factor IX concentrates |
|
|
Term
| Factor XI deficiency is aka __ __ ___ __ aka __ __ aka __ __. Physical manifestations of this deficiency are __. Bruising and petechiae NOT usually noted. No joint abnormalities usually occur. If a patient is undiagnosed, there may be prolonged bleeding/significant hematoma after surgery leading to diagnosis. |
|
Definition
- Plasma Thromboplastin Antecedent (PTA)
- Rosenthal syndrome
- Hemophilia C
- rare |
|
|
Term
| Factor XI deficiency is aka __ __ ___ __ aka __ __ aka __ __. Physical manifestations of this deficiency are __. Bruising and petechiae NOT usually noted. No joint abnormalities usually occur. If a patient is undiagnosed, there may be prolonged bleeding/significant hematoma after surgery leading to diagnosis. |
|
Definition
- Plasma Thromboplastin Antecedent (PTA)
- Rosenthal syndrome
- Hemophilia C
- rare |
|
|
Term
| Factor XI is a __ synthesized in the __ that gets activated in the plasma. Deficiency of factor XI (hemophilia C) is mainly in pple of Jewish heritage. This is an autosomal disorder. May be found after circumcision, menarche, dental procedures, or randomly with an aPTT check. Treat with ___ __ __. |
|
Definition
- zymogen
- liver
- fresh frozen plasma |
|
|
Term
| In 30% of hemophilia cases, there is no family history. These cases are likely due to spontaneous mutation. |
|
Definition
|
|
Term
| Von Willebrand Disease is a group of __ __ in which vWF is __ or __ abnormal. Usually AD rare cases of aR. |
|
Definition
- hemorrhagic disorders
- qualitatively or quantitatively |
|
|
Term
| Symptoms of Von Willebrands Disease: |
|
Definition
- pronlonged BT
- normal or low platelet count
- deficiency of factor VIII antigen
- impaired platelet adhesion |
|
|
Term
| Von Willebrands Disease represents defects in both __ _ and the __ __. |
|
Definition
- platelet function
- clotting mechanism |
|
|
Term
| these patients may lack factor VIII antigen: |
|
Definition
|
|
Term
Von Willebrand disease is an inherited bleeding disorder- meaning you are born with it.
Less than normal amounts of von Willebrand factor in the blood or von Willebrand factor that does not work as well as it should
Effects the normal ability to form a blood clot
Other parts of the clotting process do still work, however |
|
Definition
|
|
Term
A person with von Willebrand disease does not bleed any faster than normal, but may bleed for a longer time.
KNOW THIS |
|
Definition
| vWD pts don't bleed faster, just longer |
|
|
Term
| vWF is a glue like protein needed for __ __ formation. Its major functions are to serve as a bridge between __ and __ __ in the blood vessel. It also carries and protects __ __ in the blood that helps make fibrin to strengthen the plug. |
|
Definition
- platelet plug formation
- platelets and injury sites in the blood vessel
- factor VIII |
|
|
Term
von Willebrand factor (vWF) is a glue-like adhesive protein that is essential in both primary and secondary hemostasis.
In primary hemostasis, it initiates adhesion of the platelets to the injured subendothelium to form the platelet plug.
In secondary hemostasis, it serves as a carrier of factor VIII (FVIII), bringing FVIII to the site of injury where it assists in forming the fibrin clot. vWF also protects FVIII from rapid proteolytic degradation in the circulation.
Circulating vWF does not bind to platelets; however, exposure to the subendothelial matrix allows it to bind to the platelets. |
|
Definition
|
|
Term
|
Definition
Most common inherited bleeding disorder
Estimated to affect ~1% of the population (mostly type 1)
Males and females of all races may be affected
May be diagnosed at any age
Symptoms generally mild and may not require frequent treatment with type 1 |
|
|
Term
Three different types of von Willebrand Disease:
Type 1, 2 and 3
The severity of the bleeding problem will depend on the type of von Willebrand Disease |
|
Definition
Classification (major types):
Type 1, partial quantitative deficiency (≈80%)
Type 2, qualitative abnormalities (≈ 15%)
Type 3, complete deficiency (< 5%) |
|
|
Term
Type 1 vWD:
Most common type; in >80% of cases
Partial Quantitative defect
Normal structure and function of vWF
Mild to moderate decrease in amount of vWF in the blood stream
Bleeding symptoms may be absent, mild, or moderate |
|
Definition
|
|
Term
| clotting defect in pts with vWD: |
|
Definition
- platelets don't stick together as well
- fibrin is not as strong b/c less factor viii at site |
|
|
Term
Fibrin clot formation is dependent on both intrinsic and extrinsic pathways to activate the clotting factors.
Both pathways trigger the final common pathway to convert fibrinogen to fibrin. |
|
Definition
|
|
Term
| clinical presentation of vWD: |
|
Definition
- nosebleeds
- oral pharyngeal bleeds
- GI bleeding
- menorrhagia
Bleeding after surgery (especially involving mucus membranes)
Prolonged or excessive bleeding after dental procedures
Prolonged bleeding after delivery of a baby
Heavy, prolonged menstrual periods
Easy, excessive bruising
Nosebleeds
May vary in each person from time to time, throughout life |
|
|
Term
| von Willebrand Factor may be increased with: |
|
Definition
- exercise, stress
- inflammation
- pregnancy
- ocp/hrt
- smoking
- surgery, trauma, blood transfusion
- child birth |
|
|
Term
The diagnosis of type 1 vWD, the most common form of vWD, can be problematic because of the variability of the disorder.
Levels of vWF vary from day to day and can be affected by numerous factors, as indicated on this slide.
Testing at any one time may yield false-positive or false-negative results due to these factors.
Because vWF levels are lower in people with type O blood, a diagnosis of vWD should be made using laboratory ranges that are normal for this blood group. |
|
Definition
|
|
Term
|
Definition
Personal and family history of bleeding symptoms
Blood work to check von Willebrand levels and type
Type 1 disease often diagnosed later in life after extensive dental work or heavy menses.
Often adult parent diagnosed after child found to have the disease |
|
|
Term
Do you have a blood relative who has a bleeding disorder such as von Willebrand Disease or hemophilia?
Have you ever had prolonged bleeding from trivial wounds, lasting more than 15 minutes or recurring spontaneously during the 7 days after the wound?
Have you ever had heavy, prolonged or recurrent bleeding after surgical procedures, such as a tonsillectomy? |
|
Definition
Have you ever had heavy, prolonged or recurrent bleeding after dental extractions that required medical attention?
Have you ever had bleed in your stool, unexplained by a specific anatomic lesion (such an an ulcer in the stomach or a polyp in the colon) that required medical attention?
Have you ever had anemia requiring treatment or received a blood transfusion? |
|
|
Term
Most patients with type 1 vWD and some with types 2A or 2M can be treated successfully with the synthetic ___ analog, __ ___.
Frequently, bleeding symptoms are mild and treatment is not required other than conservative measures such as the application of __ and __ __ at the site of bleeding. However, prompt treatment, when required, is optimal. |
|
Definition
- vasopressin
- desmopressin acetate (DDAVP).
- ice
- firm pressure |
|
|
Term
|
Definition
Desmopressin acetate ( intravenous DDAVP, intranasal Stimate)
Intravenous Factor concentrates with von Willebrand factor and factor VIII in them
Birth control pills for heavy periods |
|
|
Term
|
Definition
- prolonged PT/APPT
- normal or low platelet count
- normal or elevated bleeding time
- multiple factor deficiencies or deficiency of common pathway |
|
|
Term
| Vitamin K is a group of fat soluble vitamins involved in the __ __ protein structural changes associated most recognizably in blood coagulation pathways but are also used in bone and other tissues. |
|
Definition
|
|
Term
K1, phylloquinone
Chloroplasts in plants
K2, menaquinone
Bacterial synthesis
K3, menadione
Synthetic, water soluble form
Complexed to improve stability. |
|
Definition
|
|
Term
K1, phylloquinone
Chloroplasts in plants
K2, menaquinone
Bacterial synthesis
K3, menadione
Synthetic, water soluble form
Complexed to improve stability. |
|
Definition
|
|
Term
| Vitamin K serves as an essential cofactor for a carboxylase that catalyzes ___ of __ __residues on __ __ __ proteins. These proteins are involved in (3): |
|
Definition
- carboxylation
- glutamic acid residues
- vitamin K dependent proteins
- coagulation
- bone mineralization
- cell growth |
|
|
Term
| Vitamin K dependent proteins: |
|
Definition
- factor II (prothrombin)
- factor VII
- factor XI (thromboplastin component)
- factor X (stuart factor)
- protein C
- protein S
- protein Z |
|
|
Term
| Warfarin inhibits __ __, thus preventing blood clotting. |
|
Definition
|
|
Term
The best food sources of vitamin K are green leafy vegetables.
Milk and liver. |
|
Definition
|
|
Term
Newborns are prone to vitamin K deficiency because…
Vitamin K and lipids are not easily transported across the placental barrier
Prothrombin synthesis in the liver is an immature process in newborns, especially when premature.
The neonatal gut is sterile, lacking the bacteria that is necessary in menaquinone synthesis.
Breast milk is not a good source of vitamin K
Results in a hemorrhagic disease called vitamin K deficiency bleeding (VKDB)
This disease is associated with breastfeeding, maladsorption of lipids, or liver
disorders. |
|
Definition
|
|
Term
| vitamin K deficiency in adults: |
|
Definition
Uncommon in adults because normal bacteria found in the gut synthesize Vitamin K2
and the vitamin may be consumed from several food sources.
Contributing Factors:
Biliary obstruction
Maladsorption
Cystic fibrosis
Resection of small intestine
Increases risk of bleeding in individuals who suffer from Vitamin K deficiency:
Coumarin anticoagulants
Certain antibiotics |
|
|
Term
| tmt of vitamin K defiency: |
|
Definition
- fresh frozen plasma
- vitamin K |
|
|
Term
| Fluoroquinolones, macrolides, etc. react with warfarin. |
|
Definition
|
|
Term
|
Definition
Decrease coagulation factor synthesis – (Factor VII)
Biliary obstruction causes impaired Vit K absorption
Portal Hypertension: hypersplenism and thrombocytopenia
Tx: factor replacement |
|
|
