321 - exam 3 p. 14-17 (DIC)

bleeding disorders resulting from a deficiency of specific coagulation proteins or clotting factor deficiencies

-It is transmitted as an X-linked recessive disorder

Hemophilia

-Pts with hemophilia do not bleed more often or even more rapidly than those without the disease, but they do bleed for a longer period.

-They form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the formation of stable fibrin clots. This allows excessive bleeding.

factor VIII (cryoprecipitate) which is needed for clotting

factor IX
- rarest also called the Christmas disease because it was discovered in a little boy in England and his last name was Christmas.

impaired platelets that adhere to damaged endothelium and deficiency factor VIII.

Missing von willebrand factor (fWF) which needs to be present in order for clotting to take place.
-stabilizes factor VII but can’t be used by the body

-avoid injury
-avoid ASA
-avoid NSAIDS

(pressure, ice)

Treatment for hemophilias

– contains all these factors (including what is needed for clotting): VIII, XIII, vWF, fibrinogen, fibronectin (usually given prior to surgery)

-clotting factors

DDAVP (desmopressin)

stimulate more factor VIII (Hemophilia A) and vWF (Von Willebrand Disease)

treatment for hemophilias

it is also called vasopressor or ADH

can be used for bed wetting (enuresis)

(increases synthesis of vWF )

-abnormal bleeding in response to trauma or surgery
-Epistaxis (nosebleeds)
-Tendency to bruise easily

degenerating joint function as a result of chronic bleeding into the joints

-joint bleeding causing pain, tenderness, swelling, and limited ROM

-prolonged PTT
-normal PT

–Shock
–Septicemia
–Hemolytic processes
–Obstetric conditions
–Tissue damage
-Trauma
-Burns

Disseminated Intravascular Coagulation

-the body starts clotting as a response to the bleeding that is occurring. The clotting cascade is activated resulting in clot formation. The rapid formation of clots causes the clotting factors and platelets to be used up so as a result of this you have more bleeding taking place and you have inefficient clotting. bleeding and clotting occur simultaneously

-uncontrolled bleeding
-bruising, purpura, petechiae, and ecchymosis

-presence of occult blood in excretions such as stool
-hematuria, hematemesis, or vaginal bleeding

all increased

decreased fibrinogen

decreased platelets (less than 100,000)

decreased

increased
- D-dimer is the end product of clot formation

decreased

-maintain urine output of 30mL/hr because renal failure is a complication of DIC

Disseminated Intravascular Coagulation

Treat cause (ex: sepsis, hemorrhage) then focus on preventing organ damage secondary to microemboli and replacing the blood clotting components) monitor for signs of microemboli (cyanotic nail beds, pain)

Heparin or LMWH (used to decrease microclots from forming and using up clotting factors) you are trying to fool the body because the problem is clotting and breakdown of clots at the same time. if you stop the clotting process, the clotting factors can accumulate and you can form a clot.

• Transfusions: Cryoprecipitate – factor VIII,
PRBC,
FFP,
platelets
– you are giving the body clotting factors so they can build up

DIC because it inhibits fibrinolysis so can enhance thrombosis (stops bleeding so clotting factors can accumulate)

-AVOID use of NSAIDS

men 4.5-5.3

women 4.1-5.1

-men 37-49%;
-women 36-46%

Hemoglobin: men 13-18; women12-16

5 -11/mm3

45-75% involved in phagocytosis

0-5% immature segs

are immature erythrocytes.

-Normal is 0.5-1.5%


(test and diagnostic book says 0.5-2)