Shared Flashcard Set

Details

Coag disorders
Pharm
17
Health Care
Graduate
12/14/2007

Additional Health Care Flashcards

 


 

Cards

Term

 

 

What deficiency in clotting factors causes hemophilia A?

Definition

 

 

 

FACTOR VIII

Term

 

 

 

What clotting factor deficiency causes

Hemophilia B?

Definition

 

 

 

Factor IX

Term

 

 

 

How are pts with Hemophilia classified?

Definition
  • Hemophilia A (factor VIII deficient)                 - most common
  • Hemophilia B (factor IX deficient)                
  • Both sex linked dxs- found on X chromosome
Term

 

 

 

Who is most commonly affected by hemophilia?

Definition

 

 

 

Men

females are carriers

Term

 

 

 

What is the normal presenting factors for hemophilia?

Definition

 

 

Bleeding episode

Term

 

 

 

How is Hemophilia A diagnosed?

Definition

   Ideally the APTT is sensitive to factor

deficiency states <30% activity.

• Patients with a normal PT but an abnormal

APTT typically have deficiencies of factors

that are unique to the intrinsic system (factors

VIII, IX, XII, and XI).

• The specific factor assay for factor VIII or IX

will reveal the appropriate defect.

• the exception is in hemophilia B
m in which the

PT and APTT are prolonged.

• ALL patients with factor VIII deficiency should undergo test for Von Willebrand’s disease.

Term

 

 

what is the treatment for Hemophilia A?

Definition
  • Those with severe or moderate disease are treated with either cryoprecipitate or factor VIII concentrate.
  • Two sources of factor VIII concentrate:                 Plasm Derived                                           Recombinant factor VIII
  • Factor is qualified in units: 1 unit of Factor VIII is same # of units in 1 ml of pooled PLTS

 

       

 

Term

 

 

What is cryoprecipitate?

Definition
  • Rich in factor VIII, fibrinogen, and Von Willibrands factor
  • each bag contains 70-100 units of factor VIII
Term

 

 

 

Desmopressin

Definition
  • used to treat mild factor VIII deficiencies
  • transiently increases factor VIII and von Willibrands factor- shortend prolonged bleeding times
  • synthetic analouge of vasopressin
Term

 

 

 

Indications for use of desmopressin?

Definition
  • treat mild factor VIII deficiencies
Term

 

 

 

Side effects of desmopressin?

Definition
  • Facial flushing (most common)
  • HA
  • Increased HR
  • Decreased BP
  • Water retention- hyponatremia
  • increases plasimogen activator
Term

 

 

Recommended treatment for pts with Inhibitors?

Definition

Antibodies to factors VIII and factor IX, also

known as inhibitors can develop in

hemophiliac patients.

• Should be suspected when calculated dose

does not result in the desired increase in

factor level.

• Treatment can be approached by different

methods

–Reduce inhibitor levels with

immunosuppresent therapy

(cyclophosphamide), gamma-globulin,

plasmopheresis, or by tolerance with

high dose or frequent administration of factor administered

During acute bleeding or

Surgery

• Patients with low titers <5 BU/ml

–Give double usual dose of factor

• Patients with high titers >10 BU/ml

–Give (PCC) Prothrombin complex

concentrate or (APCC) activated

prothrombin complex concentrate

–Porcine factor VIII Usual dose 50-75

units /Kg every 12 hours.

–Alternative is recombinant factor VIIa

Term

 

 

 

How does hemophilia Bm differ from Hemophilia B?

Definition

 

 

PT and aPTT are prolonged in Bm

Term

 

 

Recommened treatment for Hemophilia B?

Definition

• High purity factor IX plasma concentrate is

currently the treatment of choice of hemophilia B.

• Replaced older products, known as PCC with also

contained vitamin K dependant cofactors

responsible for thromboembolic complications

associated with their use.

• Half life of factor IX is 24 hours, with normal

hemostasis occuring with plasma levels are about

10-25%.

• Each unit of factor IX infused per Kg increases

factor IX plasma levels 1%

Term

 

 

 

von Willibrands Disease?

Definition
  • most common inheritable coagulopathy
  • abnormality of von Willibrands factor that results in both abnormal PLT function and deffective plasma clotting
Term

 

 

 

von Willibrands DX is classified how?

Definition
  • quantitative
  • qualitative
  • or both
Term

 

 

How is von Willibrands DX treated?

Definition
  • Goal to improve bleeding time
  • Cryoprecipitate
  • Humate-T, Koate-HS, Koate-HP
  • IV DDAVP (for mild vWD)
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