COAGULATION CASCADE THROMBOCYTOPENIA

Primary Hemostatis  Low platelet count <150,000

 Can be inherited or caused by other
1. Vasoconstriction- to decrease blood
from going to the damaged vessel.
2. Platelet adhesion (Von Wille brand
factor)
3. Activation & Degranulation –
change form via phospholipids
metabolism which produces
thromboxane
4. Platelet aggregation- recruit other
platelets

Secondary Hemostasis Intrinsic pathway-

subendothelial collagen

Extrinsic pathway- tissue factor/ thromboplastin medications or conditions

 Platelets are reduced by:
-trapping of platelets in the spleen
-↓ production
-↑ destruction of platelets
-↑ utilization of platelets

Diagnostic:

 Examining bone marrow via

aspiration & biopsy – if the platelet
destruction increased is the cause of
thrombocytopenia, the marrow
shows ↑ megakaryocytes & normal
or even ↑ platelet in production as
body attempts to compensate for ↓
platelet in circulation
 Peripheral smear

 Pseudothrombocytopenia- platelets
aggregate & clump in the presence of
EDTA acid (anticoagulant present in
the tube used for CBC collection

IMMUNE THROMBOCYTOPENIA PURPURA

-Known as Idiopathic thrombocytopenia Clinical indication: splenectomy due to portal
purpura hypertension related to cirrhosis (may cause
more bleeding disorders)
-Can be acute or chronic
Portal HPN- ↑ blood pressure within a system
-Autoimmune destruction of platelets;
of veins (stomach, liver, spleen)
antibodies destroys platelets
 Nursing Management:
-Deficiency of plasma enzyme (ADAMTS 13) that
-Bleeding precautions
breaks down VWF clotting factor
– use electric razor only
-Bleeding due to low levels of platelets

-More common among children & young

women

Pathophysiology : Idiopathic (Primary), Immune

related (Secondary)

Antibodies attach to glycoprotein IIb/IIIb, then

the macrophage will attack the platelets

Diagnostic: By exclusion, often suspected when -Avoid aspirin

platelets are extremely low or patient presents
purpura or petechiae -Protect from injury

S/Sx: asymptomatic with extremely low -Decrease use of needle sticks

platelets (single digit), bruising, heavy menses,
petechiae on extremities/ trunk, dry purpura
(bruising & petechiae), wet purpura
(hemoptysis & gingival bleeding)
Thrombocytopenia – underlying cause
 Medical management:
-Treat underlying cause: viral infection
HIV – Destruction of platelets
Disseminated Intravascular Coagulation (DIC)
-Platelet transfusion (↑ platelet count;
depends on the
-Observe for: Hematuria, melena, nosebleed)
-Administer platelets & medications as ordered
Administration of platelet depends on patient
needs (1 bag of platelet = 10,000/cu)
 Assessment
1 Check every shift if hospitalized. Notify
patient if worsening or new onset of spots
present.
Integumentary : ecchymoses, hematoma,
bleeding gums, conjuctival hemorrhage
(bleeding underneath conjunctiva

- Splenectomy – therapeutic options for other Cardiovascular: hypotension, bradycardia

patients
Pulmonary: Respiratory distress, tachypnea
Genitourinary: vaginal & urethral bleeding
Neurologic: headache, blurred vision
-Monitor lab results
-At least daily CBC platelets
-Coagulation panel
Activated Partial Thromboplastin Time
(APTT)- elevated bleeding
Normal: 2-3 seconds
-If elevated: DIC, Coagulation deficiency,
massive BT that results in dilutional
thrombocytopenia, vit. K deficiency
Notify phycian if platelets has IRSS than
10, 000/𝑚𝑚3 or has significant change from
the previous count or if has positive signs &
symptoms
-Leukocyte antigen type match
-Obtain 1hr post transfusion count if prescribed
to know its effectivity
-Obtain a test for urine, emesis, stools
 Interventions
-Avoid aspirin sulfa containing drugs or
medications that inhibit platelet aggregation –
NSAIDS, Advil, ibuprofen, aspirin
-Position client in high-fowler’s, apply ice back
to back to back of neck for bleeding. Apply
direct pressure.
Idiopathic Thrombocytopenia Purpura
 Medical Management
-Maintain safe platelet count (>30,000)
-Splenectomy
CI: Uncorrectable cardiovascular disease that
prohibits administration of anesthesia –may
cause changes in BP (Heart attack, stroke)
Side effects:
prone to infection-spleen produces
WBC
2 to 4 weeks before surgery administer
pneumovax; readminister 5-10 yrs
-Avoid sulfa containing drugs (NSAIDS,
sulfamethoxazole and trimethoprim –
(↑ platelet in few days)
Should be tapered. ↓platelets if rapid
discontinued
-Administer corticosteroids (ITP is a rare
disorder. It is administered to suppress immune
system. Corticosteriod causes bone disease)
-Give vitamin D or bioprophorate therapy to
prevent significant bone disease
-Administration of IV gamma globulin –It blocks
macrophage receptor (Fc receptor –kills
platelet)
Side effects, hypotension, back pain,
headache
-Anti D (Win-rho) for patient who are Ph D
Positive-- for patients who have protein D in
blood
-for patients who have protein D in blood
Rh D positive –universal recipient
Rh D negative –universal donor
- for patients who have protein D in blood
-given to increase platelets
-Amicar (antifibrinolytic)
 Indication: mucosal bleeding,
vincristine (antineoplastic)- blocking
receptor, macropages inhibits platelet
destruction
-Platelet transfusion-
 Nursing Management
-Assess patient’s lifestyle -determine risk for
bleeding
-Medication history –if taking sulfa containing
drugs, recent viral
illnesses & reports of headache which may be
sign of intracranial bleeding
-injections, rectal medications (taking temp in
rectum should be avoided; might cause
bleeding)
-Patient teaching: signs of exacerbation,
observe for petechia & ecchymoses, name and
type of medications to be given, how to contact
appropriate health personnel, triggers of ICP,
tapering schedule, frequency of platelet count
monitoring, bleeding precautions, refrain from
vigorous sex,
corticosteroid complications: osteoporosis,
cataract formation, dental caries
THROMBOCYTOSIS (Essential
Thrombocythemia)
-A stem cell disorder within the bone marrow
Pathophysiology: Myeloid stem cell
S/Sx:
 Thromboses (Platelet plug) in head,
hands & feet
 Bleeding (used up platelets)
 Spleenomegaly
Diagnostic: JAK2 mutation (No. of platelets
>450, 000 for ≥ 2months
Exclusion of other myeloproliferative,
inflammation, bone marrow
Medical Management
Has good prognosis ; increase risk for stroke ,
heart attack and blood cancer
 Medical Management
Cytoreductive Therapy ( reduces blood platelet
counts )
- Hydroxyurea is an antimetabolite which
is the first line of treatment ; stops
normal cells from dividing
- Bulsulfan , Analgleride – antineoplastic
agents that stops cells from producing
platelets
Heparin Therapy
Site of injections can be thighs , buttocks , 2
inches away from umbilicus
IV bolus – 60 units / kg ( 4000 units ) then 12
units / kg / hr ( 1000 units )
Aspirin – new trend : plateletpheresis
- Decreases the platelet count to safer
levels
- A special instrument is used to remove
blood from the patient . Separates and
removes platelets and retains other
blood cells to the patient.
- Its like a dialysis
Corticosteroids -blocks antiplatelet antibodies
Spleenectomy – spleen stores ; RBC , blood, -Extrinsic pathway, Intrinsic pathway,
WBC , and platelets common pathway

- Prone to infections because spleen also -Deficiency of VII [+VWF] (Hemophilia

produces WBC A): Classic
 Nursing Management
Monitor CBC with leukocyte count – may reveal -Deficiency of IX (Hemophilia B):
another underlying disease Christmas disease

Monitor for antineoplastic drugs effects –

nausea and vomiting , decrease blood count
Acquired factors:
and infections
 Liver failure- since liver
Watch out for signs of complications
synthesizes, I,II,V, VII,
- Shortness of breath VIII,IX,X,XIII
- Chest pain  Vitamin K- needed by liver
- Hemoptysis to synthesize II,VII, IX, X
- Rapid pulse  Autoimmunity against a
>> pulmonary embolism clotting factor
- Numbness of leg , arm , face of one side  Disseminated intravascular
of the body coagulation- consumes
- Blurred vision coagulation
>> stroke
- Watch out for heart attack due to S/Sx: Easy bruising (ecchymosis), hematomas
obstruction of blood clots to the flow of (collection of blood vessels often in muscles),
the heart prolonged bleeding after cut or surgery, oozing
after tooth extractions, GI bleeding, hematuria,
severe nose bleeding, hemarthrosis (bleeding
HEMOPHILIA in joint spaces), bleeding into the brain (stroke
or ↑ ICP- most severe

Diagnostic:

Platelets: Normal
-Two inherited bleeding disorders (A&B)
Prothombin time: tests extrinsic & common
-Commonly symptomatic in males, because
pathways
both types of hemophilia are inherited X-
linked traits. Females are only carriers and Normal- VII, X,V,II,I
almost always asymptomatic
PTT: tests intrinsic & common pathways
-Decreased amount or function of clotting
factor resulting more bleeding Polonged- XII, XI,IX,VIII,X,V,II,I
Confirmatory tests for specific factor Bleeding precautions:
activities & mutation testing
-If patient had recent surgery, carefully
assess sites for bleeding

Medical management -Administer analgesic as required –for

Infusion of fresh frozen plasma – contains all pain
coagulant factors
-Allow warm baths but avoid during
-270 to 380ml/ bag as much as possible
bleeding episodes –relaxation
-Factor VIII and IX administration – large
-Advise patient to wear medical identification
quantities are needed ; short half life
band
-Preventive measures before traumatic
procedure (lumbar puncture)
DISSEMINATED INTRAVASCULAR
-Plasmapharesis /concurrent
immunosuppressive therapy – for patients who
COAGULATION
developed antibodies (inhibitors) to factor
-Not a disease but a sign of an underlying
concentration (destroys transfused platelets)
condition
-Aminocaproic acid – unsuccessful usually
-May be triggered by sepsis, trauma,
mucosal bleeding
cancer, shock, abruption placenta, toxic,
-Desmopressin – increased in factor VIII allergic, reactions & other conditions
-not effective if severe
-OB: DIC occurs when there is such extreme
 Nursing Management bleeding & so many platelets and fibrin
-Assist family in coping from circulation rush to the site that there
place restriction, and activities to
is not enough left in the rest if the body
avoid

Recommend genetic testing because it

is inherited Patho:
-From childhood help identify positive aspects
of their lives
Bleeding
-Avoid medications that aggregate platelet
production

Alcohol, nutritional supplement, NSAIDS Primary

hemostasis
-Promote good dental hygiene –preventive
measure for dental extractions
Fibrinogen (Secondary
Hemostasis)
Secondary  Medical Management
Treating underlying cause

Can be infection , cardiac arrest or OB

Formation of Procoagulants are complications
hard clot Released that ↑clot *Cardiac arrest activates coagulation -> excess
(Widespread clot formation
circulation thrombin -> blockage of blood
formation
Complication: vessels by clot -> clot obstruction leads to
ischemia, necrosis, hemorrhage.
organ damage
Prevent and treat serious hemorrhage
Clot breakdown
(Fibrinolysis) Replacement of deficient clotting factors –
platelet transfusion to normalize hemostatis

Degradation Fibrin degradation Cryoprecipitate

products products in
Heparin transfusion – the use of heparin in the
circulation
treatment of DIC remains controversial
interferes with clot
formation Heparin interrupts thrombosis and inhibit
↓ Supply of formation of mincrothrombi and permi
platelets & perfusion of organs ( kidney , skin , brain ) to
clotting factors resume

Contraindicated in patients whom with

thrombotic manifestations , failed blood
transfusions( anticoagulant [ antibodies ] vs
coagulant platelets )

For effectiveness :

- Check for normal plasma ( fibrinogen

Labs:
 ↓ platelet & ↓ Fibrinogen
 Prolonged PT & PTT – reflect ↓
circulating coagulation factors
 ↑ D-Dimer D Fibrin degradation
product
 Chronic DIC – compensation
(normal results)
concentration and diminished signs of
bleeding )
- Amicar may be used with heparin
Indicated for patients with slow evolving DIC
who have venous thromboembolism
Recombinant Activated C-Protein
administration – to diminish inflammatory
response on the surface of the blood vessel
Antithrombin Infusions – for anticoagulation
and anti-inflammation properties
  Nursing Management
Maintaining Hemodynamics by :

- Avoid coughing and straining -to avoid

increase ICP
- Avoid medications that interfere with
platelet function ( ex. Betalactam ,
NSAID , ASA )
- Perform oral hygiene
Maintaining Skin Integrity

- Apply pressure for 5 minutes after

injection
Monitoring for Fluid Volume Imbalance

- Auscultate/ Assess breath sounds every

2-4 hours ( crackles develop quickly )
- Check for Edema ( fluid extends beyond
intravascular system )
- Monitor volume of intravenous
medications ( to prevent fluid overload
)
Assess for Ineffective tissue perfusion

- Monitor response to heparin therapy (

monitor fibrinogen )
- Stop epsilon aminocaproic acid ( if
thrombosis occurs )
Reducing fear and anxiety

- Support patient and the family( coping

mechanism and use of services from
behavioral science )