Bleeding DisordersAcquired Coagulation Disorder

Republic of the Philippines
JH CERILLES STATE COLLEGE

Pagadian City Campus
West Capitol Road, Balangasan District, Pagadian City
In consortium with
Western Mindanao State University
Zamboanga City
A Case Study on Bleeding Disorder and Acquired Coagulation Disorder
In Partial Fulfillment of
the Requirements for the NCM 116
MEDICAL-SURGICAL NURSING
Submitted by:
Joanna Yvonne A. Dela Luna
BSN-III, Batch Merakia
Submitted to:
Donna Mae R. Dumaog, MN, MAN
2nd Semester, A.Y. 2020-2021

School of Arts & Sciences

BACHELOR OF SCIENCE IN NURSING
Bleeding Disorders
Failure of hormonal hemostatic mechanism can result in bleeding, which may be severe.
Bleeding is commonly provoked by trauma; however in certain circumstances, it can occur
spontaneously. When the cause is platelet or coagulation factor abnormalities, the site of
bleeding ca be anywhere in the body. When the source is vascular abnormalities, the site of
bleeding may be localized. Some patients have simultaneous defects in more than one
hemostatic mechanism.
The bone marrow may be stimulated to increase platelet production (thrombopoiesis). This
may be reactive response, as in a compensatory response to significant bleeding, or more
general response to increased hematopoiesis, as in iron deficiency anemia. The spleen
typically holds about one third of the circulating platelets at any time. If spleen is absent, the
platelet reservoir is also lost, and an abnormally high number of platelets enter the
circulation. In time, the rate of thrombopoiesis slows to reestablish a more normal platelet
level.
The majority of bleeding disorders are inherited, which means they’re passed from a parent
to their child. However, some disorders may develop as a result of other medical conditions,
such as liver disease.
Bleeding disorders may also be caused by:
 a low red blood cell count

 a vitamin K deficiency
 side effects from certain medications
Signs and symptoms
The symptoms can vary depending on the specific type of bleeding disorder. However, the
main signs include:
 unexplained and easy bruising

 heavy menstrual bleeding
 frequent nosebleeds
 excessive bleeding from small cuts or an injury
 bleeding into joints
Complications often occur when bleeding disorders are treated too late.
Clinical Instructor: Donna Mae R. Dumaog, MN, MAN Student Nurse: Joanna Yvonne A. Dela Luna
NCM 116 – Medical-Surgical Nursing


Common complications of bleeding disorders include:
 bleeding in the intestines

 bleeding into the brain
 bleeding into the joints
 joint pain
Complications can also arise if the disorder is severe or causes excessive blood loss
Tests
 Complete blood count (CBC)

Your doctor may order a complete blood count (CBC) as part of your routine physical.
The results of the test can alert your doctor if you have anemia or a low platelet count,
which can interfere with your ability to clot.
 Factor V assay
This test measures Factor V, a substance involved in clotting. An abnormally low level
may be indicative of liver disease, primary fibrinolysis (a breakdown of clots),
or disseminated intravascular coagulation (DIC).
 Fibrinogen level
Fibrinogen is a protein made by your liver. This test measures how much fibrinogen is in
your blood. Abnormal results may be a sign of excessive bleeding or hemorrhage,
fibrinolysis, or placental abruption, which is a separation of the placenta from the uterine
wall.
Other names for this test include factor I and hypofibrinogenemia test.
 Prothrombin time (PT or PT-INR)
Prothrombin is another protein your liver produces. The prothrombin time (PT) test
measures how well and how long it takes your blood to clot. It normally takes about 25 to
30 seconds. It may take longer if you take blood thinners. Other reasons for abnormal
results include hemophilia, liver disease, and malabsorption. It’s also useful in monitoring
those who take medications that affect clotting, such as warfarin (Coumadin).
 Platelet count
Platelets are cells in the blood that help your blood clot. You may have an abnormally low
number if you’re on chemotherapy, take certain medications, or have had a massive blood
transfusion. Other causes of a low platelet count are celiac disease, vitamin K deficiency,
and leukemia.
 Thrombin time
Thrombin time measures how well fibrinogen is working. Abnormal results may be due to
inherited fibrinogen disorders, liver disease, some cancers, and medications that affect
clotting.
 Bleeding time
This test analyzes how quickly small blood vessels in your skin close up and stop
bleeding. It’s performed differently than the other blood tests.


A blood pressure cuff will be placed on your upper arm and inflated. Your healthcare
provider will make a couple of tiny cuts on your lower arm. The cuts won’t be deep and will
generally feel like scratches.
Your healthcare provider will remove the cuff when it’s deflated and briefly place blotting
paper on the cuts every 30 seconds until bleeding stops.
Bleeding usually lasts between one to nine minutes. The test is considered safe and carries
few side effects or risks.
Secondary Thrombocytosis
Increased platelet production is the primary mechanism of secondary, or reactive

thrombocytosis. The platelet count is above normal, but, in contrast to essential
thrombocythemia, an increase of more than 1 million/mm3 is rare. Platelet function is
normal; the platelet survival time is normal or decreased. Consequently, symptoms
associated with hemorrhage or thrombosis are rare. Many disorder or conditions can cause a
reactive increase in platelets, including infection, iron deficiency, chronic inflammatory
disorders, malignant disease, acute hemorrhage, and splenectomy.
Thrombocytopenia
Thrombocytopenia (low platelet level) can result from various factors: decreased production
of platelets within the bone marrow, increased destruction of platelets, or increased
consumption of platelets.
Signs and Symptoms

Thrombocytopenia signs and symptoms may include:
 Easy or excessive bruising (purpura)

 Superficial bleeding into the skin that appears as a rash of pinpoint-sized reddish-purple
spots (petechiae), usually on the lower legs
 Prolonged bleeding from cuts
 Bleeding from your gums or nose
 Blood in urine or stools
 Unusually heavy menstrual flows
 Fatigue
 Enlarged spleen
Risk factors


 Have certain types of cancer, aplastic anemia, or autoimmune diseases

 Are exposed to certain toxic chemicals
 Have a reaction to certain medicines
 Have certain viruses
 Have certain genetic conditions
Complications
 Prolonged bleeding, even from minor cuts

 Bleeding or oozing from the mouth or nose, especially nosebleeds or bleeding from
brushing your teeth
 Abnormal vaginal bleeding (especially heavy menstrual flow)
Management
Nursing Management
 minimize the patient’s risk of injury, as by preventing falls, reducing the bleeding risk,
and preventing infection
 Monitor the patient’s fluid intake and output to evaluate hydration status and check stools
for occult blood
 To minimize the bleeding risk, restrict the use of venipuncture, razors, toothbrushes,
dental floss, tampons, and intramuscular and subcutaneous injections
 If the patient requires isolation due to immunosuppressants or infection, perform a
psychosocial assessment to evaluate psychosocial needs
 Determine the types of assistance the patient may need with activities of daily living
Medical Management
 Treatment for thrombocytopenia depends on its cause and severity. The main goal of
treatment is to prevent death and disability caused by bleeding.
 If your condition is mild, you may not need treatment. A fully normal platelet count isn't
necessary to prevent bleeding, even with severe cuts or accidents.
 If your immune system is causing a low platelet count, your doctor may prescribe
medicines to suppress the immune system.
 Steroids may slow platelet destruction. These medicines can be given through a vein or
by mouth. One example of this type of medicine is prednisone.
 Immunoglobulins or medicines like rituximab to block your immune system. These
medicines are given through a vein
 Eltrombopag or romiplostim, to help your body make more platelets
 Blood or platelet transfusions are used to treat people who have active bleeding or are at
a high risk of bleeding
Surgical Management
 Splenectomy is surgery to remove the spleen. This surgery may be used if treatment with
medicines doesn't work.


Immune Thrombocytopenic Purpura
Immune Thrombocytopenic Purpura (ITP) is a disease that affects people of all ages, but is
more common among children and young women. Other names for the disorder are
idiopathic thrombocytopenic purpura and immune thrombocytopenia. Primary ITP occurs in
isolation. Secondary ITP often results from autoimmune diseases (e.g., antiphospholipid
antibody syndrome), viral infections (e.g., Hepatitis C,HIV) and various drugs (e.g., sulfa
drugs)
Signs and Symptoms
 Easy or excessive bruising (purpura)

 Superficial bleeding into the skin that appears as a rash of pinpoint-sized reddish-purple
spots (petechiae), usually on the lower legs
 Bleeding from your gums or nose
 Blood in urine or stools
 Unusually heavy menstrual flows
 Fatigue
 Enlarged spleen
Risk factors
 ITP is more common among young women.

 Diseases such as rheumatoid arthritis, lupus and antiphospholipid syndrome.
Complications
 A rare complication of immune thrombocytopenia is bleeding into the brain, which can
be fatal.
 If you're pregnant and your platelet count is very low or you have bleeding, you have a
greater risk of heavy bleeding during delivery.
Pathophysiology
ITP is an autoimmune disorder characterized by a destruction of normal platelets by an

unknown stimulus. Antiplatelet antibodies develop in the blood and bind to the patient’s
platelets. These antibody-bound platelets are then ingested and destroyed by the
reticuloendothelial system ( RES) or tissue macrophages. The body attempts to compensate
for this destruction by increasing platelet production within the marrow. However, platelet


production may also be impaired as the antibodies may also induce cell death (via apoptosis)
of the megakaryocytes and thus inhibit platelet production within the bone marrow.
Destruction of normal platelets by unknown stimulus
Antiplatelets antibodies develop in the blood and

bind to patient’s platelet
Ingested and destroyed by the reticuloendothelial

system (RES) or tissue macrophages
Increase platelet production within the marrow
The antibodies may also induce cell death (via

apoptosis) of the megakaryocytes
Impaired platelet production
Management
Nursing Management
 Assessment of the patient’s lifestyle to determine the risk of bleeding from activity
 Nurse must be alert of sulfa-containing medications and others that alter platelet function
 Nurse assesses for any history of recent viral illnesses and reports headache or visual
disturbances, which could be initial symptoms of intracranial bleeding
 All injections or rectal medications should be avoided, and rectal temperature measurements
should not be performed, because they can stimulate bleeding
 Nurses should explore the extent the patient experiences fatigue and offer strategies to
ameliorate this problem
 Bone mineral density should be monitored


Medical Management
Medications to treat ITP may include:
 Steroids. Your doctor will likely start you on an oral corticosteroid, such as prednisone.
Once your platelet count is back to a safe level, you can gradually discontinue taking the
drug under the direction of your doctor. Long-term use of these medications is not
recommended because they can increase your risk of infections, high blood sugar and
osteoporosis.
 Immunoglobulin. If corticosteroids don't help, your doctor may give you an injection of
immune globulin. This drug may also be used if you have critical bleeding or need to quickly
increase your blood count before surgery. The effect usually wears off in a couple of weeks.
 IVIG is also commonly used to treat ITP. It is effective in binding the receptors on the
macrophage; however, high doses are required, it is very expensive, and effect is transient
 Use of anti-D (WinRho) in patients who are Rh (D) positive. Actual mechanism is
unknown. One theory is that it binds to the patient’s erythrocytes, which are in turn destroyed
by the body’s macrophages
Surgical Management
 Splenectomy is surgery to remove the spleen. This surgery may be used if treatment with
medicines doesn't work.
Platelet Defects
Quantitative platelet defects are relatively common; however, qualitative defects can also
occur. With qualitative defects, the number of platelets may be normal but the platelets do
not function normally. A platelet function analyzer is used to evaluate platelet function; this
method is particularly valuable for rapid and simple screening. Examining the platelet
morphology of platelets is often hypogranular and pale, and may be larger than normal.
Aspirin may induce platelet disorder. Even small amounts of aspirin reduce normal platelet
aggregation, and the prolonged bleeding time lasts for several days after aspirin ingestion.
Although this does not cause in most people, patients with a coagulation disorder or
thrombocytopenia can have significant bleeding after taking aspirin, particularly if invasive
procedures or trauma have occurred.
NSAIDs can also inhibit platelet function, but the effect is not as prolonged as with aspirin
(about 4 days vs 7 to 10 days).
Other causes of platelet dysfunction include end-stage renal disease, possibly from metabolic
products affecting platelet function; MDS; multiple myeloma (due to abnormal protein


interfering with platelet function); cardiopulmonary bypass; herbal therapy; and other
medications.
Signs and symptoms
 Severe and/or mild bleeding

 Ecchymoses are common particularly on the extremities
 Bruising
 petechiae (pinprick bleeds in the skin),
 bleeding gums
 heavy menstrual periods
Management
Nursing Management
 Instruct patient to avoid substances that can diminish platelet function, such as certain
OTC medications, some herbal therapies, nutritional supplements and alcohol
 Maintain good oral hygiene
 Encourage patient to inform their health care providers of the underlying condition
before any invasive procedure is performed so that appropriate stps can be initiated to
diminish the risk of bleeding
Medical Management
 If platelet dysfunction is caused by medication, its use should be stopped,if possible,
particularly when bleeding occurs.
 Antifibrinolytic agents (e.g., aminocaproic acid) may be required to prevent significant
bleeding after such procedures
 Desmopressin (DDAVP) can decrease the duration of bleeding in some situations and
improve hemostasis
Hemophilia
Two inherited bleeding disorders-hemophilia A and hemophilia B- are clinically

indistinguishable, although they can be distinguished by laboratory tests. Hemophilia A is
caused by genetic defect that results in deficient or defective factor VII. Hemophilia B (also
called Christmas disease) stems from a genetic defect that causes deficient or defective
factor XI.
Both types of hemophilia are inherited as X-linked traits, so most affected people are males;
females can be carriers but are almost always asymptomatic. The disease occurs in all ethnic
groups.
The tendency for developing bleeding serves as the basis for hemophilia classification:
 Severe disease is defined as a plasma factor activity level of less than 1 IU/dL, or less
than 1% normal factor VIII levels


 Moderate disease reflects a level of 1-5 IU/Dl or factor VIII level between 1% and 5% of
normal
 Mild disease reflects a level above 5 IU/dL or factor VIII level above 5%
Hemophilia is recognized in early childhood, usually in the toddler age group. However,
patients with mild hemophilia may not be diagnosed until they experience trauma or surgery.
Signs and symptoms
 Hemorrhage into various parts of the body

 Pain in joints ( knees, elbows, ankles, shoulders, wrists and hips)
 Chronic pain
 Decreased sensation
 Weakness
 Atrophy in area involved
Pathophysiology
Hemophilia is an X-linked recessive genetic disease causing abnormal bleeding because of a

specific clotting factor malfunction. Factors VIII and IX are components of the intrinsic
clotting pathway; factor IX is an essential factor and factor VIII is a critical cofactor that
accelerates the activation of factor X by several thousandfold. Excessive bleeding occurs
when these clotting factors are reduced by more than 75%. A deficiency or nonfunction of
factor VIII causes hemophilia A, and a deficiency or nonfunction of factor IX causes
hemophilia B.
Hemophilia may be severe, moderate, or mild, depending on the degree of activation of

clotting factors. Patients with severe disease have no detectable factor VIII or factor IX
activity. Moderately afflicted patients have 1% to 4% of normal clotting activity, and mildly
afflicted patients have 5% to 25% of normal clotting activity.
A patient with hemophilia forms a platelet plug at a bleeding site, but clotting factor
deficiency impairs the ability to form a stable fibrin clot. Bleeding occurs primarily into large
joints, especially after trauma or surgery. Delayed bleeding is more common than immediate
hemorrhage. Spontaneous intracranial bleeding may be fatal.
Activation of Coagulation
Excess of circulating thrombin Positive feedback


Blood vessel blockage by fibrin Organ failure

clots
Consumption of platelets and
Clot destruction by fibrinolysis
factors
Hemorrhage
Diagnostic test results
 Specific coagulation factor assays show the type and severity of hemophilia.
 Laboratory analysis reveals low serum factor VIII activity of normal and prolonged
activated partial thromboplastin time (hemophilia A).
 Laboratory analysis reveals deficient factor IX and normal factor VIII levels (hemophilia
B).
Treatment
 Cryoprecipitate (for hemophilia A) or lyophilized factor VIII or IX to increase clotting

factor levels and to permit normal hemostasis levels
 Factor IX concentrate during bleeding episodes (hemophilia B)
 Aminocaproic acid (Amicar) for oral bleeding (inhibits plasminogen activator
substances)
 Prophylactic desmopressin before dental procedures or minor surgery to release stored
von Willebrand's factor and factor VIII (to reduce bleeding)
 Fresh frozen plasma
 Immunization with hepatitis B vaccine
Management
Nursing Management


 Patients are encouraged to be self-sufficient and to maintain independence by preventing

unnecessary trauma that can cause acute bleeding episodes and temporarily interfere with
normal activities.
 Patients need extensive education about activity restrictions and self-care measures
 Patients and family members are instructed how to administer the factor concentrate ate
home at the first sign of bleeding
 Patients are instructed to avoid agents that interfere with platelet aggregation such as
aspirin , NSAIDs, some herbal and nutritional supplements
 Oral hygiene is very important as a preventive measure
Medical Management
 Recombinant forms of factor VIII and X concentrates are available and decrease the need
for using factor concentrates or more infrequently, fresh-frozen plasma
 Children typically receive factor administration prophylactically, three to four times each
week
 Patients with severe factors deficiency should be screened for antibodies
 Aminocaproic acid inhibits fibrinolysis and therefore stabilizes the clot
 In patients with mild forms of hemophilia A, desmopressin is extremely useful,
significantly reducing the amount of blood products required
 Desmopressin is not effective in patients with severe factor VIII deficiency
Von Willebrand Disease (vWD)
Usually inherited as a dominant trait, vWD is a common bleeding disorder that affects males
and females equally. The prevalence of this disease is estimated to be 1% or 2% of the
population. The disease is caused by a deficiency of vWF , which is necessary for factor VIII
activity. vWF is also necessary for platelet adhesion at the site of vascular injury. Although
synthesis of factor VIII is normal, its half-life is shortened; therefore, factor VIII levels
commonly are mildly low.
There are three types of vWD:
Type 1: the most common, is characterized by decrease in structurally normal vWF.

Type 2: shows variable qualitative defects based on the specific vWF subtype involved
Type 3: is very rare (less than 5% of cases) and is characterized by a severe vWF deficiency
as well as significant deficiency of factor VIII
Signs and symptoms
 Recurrent nosebleed
 Easy bruising
 Heavy menses
 Postoperative bleeding


Assessment and Diagnostic Findings
The diagnosis is established by the patient meeting all this criteria:

 A history of bleeding since childhood
 Reduced vWF activity in plasma
 History of bleeding within the family
 Ristocentin cofactor
 vWF collagen binding assay
 Von Willebrand factor antigen. This determines the level of von Willebrand factor in
your blood by measuring a particular protein.
 Von Willebrand factor activity. There are a variety of tests to measure how well the
von Willebrand factor works in your clotting process.
 Factor VIII clotting activity. This shows whether you have abnormally low levels and
activity of factor VIII.
 Von Willebrand factor multimers. This evaluates the structure of von Willebrand
factor in your blood, its protein complexes and how its molecules break down. This
information helps identify the type of von Willebrand disease you have.
Management
Your doctor might suggest one or more of the following treatments to increase your von
Willebrand factor, strengthen blood clots or, in women, control heavy menstrual bleeding:
 Desmopressin. This medication is available as an injection (DDAVP) or nasal spray

(Minirin) It's a synthetic hormone that controls bleeding by stimulating your body to
release more of the von Willebrand factor stored in the lining of your blood vessels.
Many doctors consider DDAVP the first treatment for managing von Willebrand disease.
Some women use the nasal spray at the beginning of their menstrual periods to control
excessive bleeding. It can also be effective when used before a minor surgical procedure.
 Replacement therapies. These include infusions of concentrated blood-clotting factors
containing von Willebrand factor and factor VIII. Your doctor might recommend them if
DDAVP isn't an option for you or was ineffective.
Another replacement therapy approved by the FDA for treating adults 18 and older is a
genetically engineered (recombinant) von Willebrand factor product. Because
recombinant factor is made without plasma, it can reduce the risk of a viral infection or
allergic reaction.
 Oral contraceptives. For women, these can be useful for controlling heavy bleeding
during menstrual periods. The estrogen hormones in birth control pills can boost von
Willebrand factor and factor VIII activity. This effect is likely available with birth
control patches, though further study is needed to confirm it.
 Clot-stabilizing medications. These anti-fibrinolytic medications — such as
aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda) — can help


stop bleeding by slowing the breakdown of blood clots. Doctors often prescribe these
drugs before or after a surgical procedure or tooth extraction.
 Drugs applied to cuts. A fibrin sealant (Tisseel VHSD) placed on a cut helps curtail
bleeding. This is applied like glue using a syringe. There are also over-the-counter
products to stop nosebleeds.
Discharge Plan
 Medications
- Instruct significant others to timely follow the ordered prescription and to strictly
monitor the right time, dose, frequency and route in giving the medications.
 Environment
- Encourage significant others to maintain the cleanliness of the house and surrounding.
- Refer patient to home care nurse, and assist in transition from hospital to home.
 Exercise/ Activity
- Encourage rest and probably a change in lifestyle (adequate, well-balanced diet and
elimination of alcohol).
- Advice to avoid doing strenuous activities.
 Treatment
- Instruct patient to comply medication.
- Encourage patient to go to follow up consultation on the prescribe date.
 Health Teaching
- Provide written instructions, teaching, support, and reinforcement to patient and family.
- Observe signs and symptoms that need reporting.
- Encourage patient and s/o to observe condition and if any unusual health problem to the
health care provider.
 Outpatient/Inpatient Referrals
- Advise to avoid activities that may cause scratches or bruises and use caution with skin
and mouth care
 Diet
- Encourage patient to have a healthy diet.
- Encourage patient to increase fluid and electrolyte intake.
 Spiritual
- Encourage patient to have a strong spiritual connection.


ACQUIRED OAGULATION DISORDERS
Liver Disease
With the exception of factor VIII, most blood coagulation factors are synthesized in the liver.
Therefore, hepatic dysfunction (due to cirrhosis, tumor, or hepatitis) can result in diminished
amounts of the factors needed to maintain coagulation and hemostasis. Prolongation of the
PT, unless it is caused by vitamin K deficiency, may indicate severe hepatic dysfunction.
Although bleeding is usually minor, these patients are also at risk for significant bleeding,
related especially to trauma or surgery. Transfusion of fresh-frozen plasma may be required
to replace clotting factors and to prevent or stop bleeding. Patients may also have life-
threatening hemorrhage from peptic ulcers or esophageal varices. In these cases, replacement
with fresh-frozen plasma, PRBCs, and platelets is usually required.
Signs and Symptoms
If signs and symptoms of liver disease do occur, the may include:
 Skin and eyes that appear yellowish (jaundice)

 Abdominal pain and swelling
 Swelling in the legs and ankles
 Itchy skin
 Dark urine color
 Pale stool color
 Chronic fatigue
 Nausea or vomiting
 Loss of appetite
 Tendency to bruise easily


Risk Factor
Factors that may increase your risk of liver disease include:
 Heavy alcohol use

 Obesity
 Type 2 diabetes
 Tattoos or body piercings
 Injecting drugs using shared needles
 Blood transfusion before 1992
 Exposure to other people's blood and body fluids
 Unprotected sex
 Exposure to certain chemicals or toxins
 Family history of liver disease
Complications
Complications of liver disease vary, depending on the cause of your liver problems.
Untreated liver disease may progress to liver failure, a life-threatening condition.
Prevention
To prevent liver disease:
 Drink alcohol in moderation. For healthy adults, that means up to one drink a day for
women and up to two drinks a day for men. Heavy or high-risk drinking is defined as
more than eight drinks a week for women and more than 15 drinks a week for men.
 Avoid risky behavior. Use a condom during sex. If you choose to have tattoos or body
piercings, be picky about cleanliness and safety when selecting a shop. Seek help if you
use illicit intravenous drugs, and don't share needles to inject drugs.
 Get vaccinated. If you're at increased risk of contracting hepatitis or if you've already
been infected with any form of the hepatitis virus, talk to your doctor about getting the
hepatitis A and hepatitis B vaccines.
 Use medications wisely. Take prescription and nonprescription drugs only when
needed and only in recommended doses. Don't mix medications and alcohol. Talk to your
doctor before mixing herbal supplements or prescription or nonprescription drugs.


 Avoid contact with other people's blood and body fluids. Hepatitis viruses can be
spread by accidental needle sticks or improper cleanup of blood or body fluids.
 Keep your food safe. Wash your hands thoroughly before eating or preparing foods. If
traveling in a developing country, use bottled water to drink, wash your hands and brush
your teeth.
 Take care with aerosol sprays. Make sure to use these products in a well-ventilated
area, and wear a mask when spraying insecticides, fungicides, paint and other toxic
chemicals. Always follow the manufacturer's instructions.
 Protect your skin. When using insecticides and other toxic chemicals, wear gloves,
long sleeves, a hat and a mask so that chemicals aren't absorbed through your skin.
 Maintain a healthy weight. Obesity can cause nonalcoholic fatty liver disease.
Vitamin K Deficiency
The synthesis of many coagulation factors depends on vitamin K. Vitamin K deficiency is
common in malnourished patients. Prolonged use of some antibiotics decreases the intestinal
flora that produces vitamin K, depleting vitamin K stores. Administration of vitamin K
(phytonadione [Mephyton]) either orally or as a subcutaneous injection, can correct the
deficiency quickly; adequate synthesis of coagulation factors is reflected by normalization of
the PT.
Signs and symptoms

The signs and symptoms associated with vitamin K deficiency may include:
 bruises easily
 gets small blood clots underneath their nails
 bleeds in mucous membranes that line areas inside the body
 produces stool that looks dark black (almost like tar) and contains some blood
In infants, doctors may observe vitamin K deficiency if there is:
 bleeding from the area where the umbilical cord is removed

 bleeding in the skin, nose, the gastrointestinal tract, or other areas
 bleeding at the penis if the baby has been circumcised
 sudden bleeding in the brain, which is extremely dangerous and life-threatening
Risk Factors


 take coumarin anticoagulants such as warfarin, which thins the blood

 are taking antibiotics
 have a condition that causes the body to not absorb fat properly (fat malabsorption)
 have a diet that is extremely lacking in vitamin K
Tests
 A deficiency of vitamin K is usually discovered when unexpected or excessive bleeding

occurs. In such cases, a prothrombin time (PT/INR) is the main laboratory test performed
to investigate the bleeding
 partial thromboplastin time (PTT),
 Platelet count
 platelet function tests
 coagulation factor tests
Disseminated Intravascular Coagulation

Disseminated intravascular coagulation (DIC) is not an actual disease but a sign of an
underlying condition. DIC may be triggered by sepsis, trauma, cancer, shock, abruptio
placentae, toxins, allergic reactions, and other conditions; the vast majority (two thirds) of
cases of DIC are initiated by an infection or malignancy. The severity of DIC is variable, but
it is potentially life-threatening.
Signs and symptoms

Symptoms of DIC may include any of the following:
 Bleeding, from many sites in the body.

 Blood clots.
 Bruising.
 Drop in blood pressure.
 Shortness of breath.
 Confusion, memory loss or change of behavior.
 Fever.
Risk factors for DIC include:
 Blood transfusion reaction

 Cancer, especially certain types of leukemia
 Inflammation of the pancreas (pancreatitis)


 Infection in the blood, especially by bacteria or fungus

 Liver disease
 Pregnancy complications (such as placenta that is left behind after delivery)
 Recent surgery or anesthesia
 Severe tissue injury (as in burns and head injury)
 Large hemangioma (a blood vessel that is not formed properly)
Pathophysiology
In DIC, normal hemostatic mechanism are related. The inflammatory response generated by
the underlying disease initiates the process of inflammation and coagulation within the
vasculature. The natural anticoagulant pathways within the body are simultaneously
impaired, and the fibrinolytic system is suppressed so that a massive amount of tiny clots
forms in the microcirculation. Initially, the coagulation time is normal. However, as the
platelets and clotting factors form microthrombi, coagulation fails. Thus the paradoxical
result includes excessive clotting and bleeding.
The clinical manifestation of DIC are primarily reflected in compromised organ function or
failure. Decline in organ function usually a result of excessive clot formation (with resultant
ischemia to all or part of the organ), or less often, of bleeding. The excessive clotting triggers
the fibrinolytic system to release fibrin degradation products, which are potent
anticoagulants, furthering the bleeding. The bleeding is characterized by low platelet and
fibrinogen levels; prolonged PT, aPTT, and thrombin time; and elevated fibrin degradation
products and D-dimers.
The mortality rate can exceed 80% in patients who develop severe DIV with ischemic
thrombosis, frank hemorrhage and multiple organ dysfunction syndrome (MODS).
Identification of patients who are at risk for DIC and recognition of the early clinical
manifestation of this syndrome can result in prompt medical intervention, which may
improve the prognosis. However, the primary prognostic factor is the ability to treat the
underlying condition that precipitated DIC.
Significant illness
(Sepsis, trauma, certain malignancy, complications from

pregnancy)
Pathological activation of coagulation and Activation of abnormal fibrinolysis

impaired fibrinolysis (initially)
(later)
Thrombus formation in
microcirculation Further bleeding


Consumption of platelets and

Tissue ischemia
clotting factors
Bleeding End organ damage
End organ failure
Management
Nursing Management
 Patients need to be assessed thoroughly and frequently for signs and symptoms of
thrombi and bleeding and monitor for any progression of these signs
 Lab values must be monitored frequently, not only for the actual result but to note
trends over time as well as the rate of change in values
 Assessment and intervention should target potential sites of end-organ damage
 Respiratory function warrants careful monitoring and aggressive measures to diminish
alveolar compromise.
 Suctioning should be performed as gently as possible to diminish the risk of additional
bleeding
Medical Management
 Cryoprecipitate is given to replace fibrinogen and factors V and VII
 Administering fresh frozen plasma replaces coagulation factors but can exacerbate
capillary leak further compromising pulmonary function
 Platelets are transfused to correct severely low platelet level, control bleeding, or prior to
an invasive procedure.
 Controversial treatment strategy is to interrupt the thrombosis process through the use of
heparin infusion
 Prophylactic doses of unfractionated heparin or LMWH may be used to prevent venous
thromboembolism
 Fibrinolytic inhibitors, such as aminocaproic acid are not routinely advised; they block
the lysis of fibrin needed to preserve tissue perfusion.


Thrombotic Disorders
Several conditions can alter balance within the normal hemostasis process, causing excessive
thrombosis that may be arterial (due to platelet aggregation) or venous (composed of
platelets, red cells, and thrombin). Abnormalities that predispose a person to thrombotic
events include decreased clotting inhibitors within the circulation (which enhances
coagulation), altered hepatic function (which may decrease the production of clotting factors
or clearance of activated coagulation factors), lack of fibrinolytic enzymes, and tortuous or
atherosclerotic vessels (which promotes platelet aggregation). Thrombosis may occur as an
initial manifestation of an occult malignancy or as a complication from a pre-existing cancer.
It can also be caused by more than one predisposing factor. Several inherited or acquired
deficiency conditions, including hyperhomocysteinemia, antithrombin (AT) deficiency,
protein C deficiency, protein S deficiency, activated protein C (APC) resistance, and factor V
Leiden deficiency can predispose a patient to a repeated episodes of thrombosis; they are
referred to as hypercoagulable states of thrombophilia. Those disorders that are inherited
should trigger the need for familial genetic testing disorder; acquired disorders do not
warrant familial testing.
Conditions that may result from thrombosis include acute coronary syndrome (ACS),
ischemic stroke, and peripheral arterial occlusive disease. Anticoagulation therapy is
necessary. The duration of therapy varies with the location and extent of thrombosis,
precipitating events, and concurrent risk factors. A recent study found that taking aspirin
after completing standard anticoagulation therapy for treating VTE reduced the risk of
recurrent thrombosis.
Hyperhomocysteinemia
Homocysteine can promote platelet aggregation. Increased plasma levels of homocysteine
are a significant risk factor for VTE, pulmonary embolism (e.g., deep vein thrombosis
[DVT], pulmonary embolism [PE]), recurrent VTE, and arterial thrombosis.
Hyperhomocysteinemia can be hereditary, or it can result from a nutritional deficiency of
folate, and, to a lesser extent, of vitamins B12 and B6, because these vitamins are cofactors
in homocysteine metabolism. For unknown reason people who are older and those with
kidney injury may also have elevated levels of homocysteine in the absence of nutritional
deficiencies of these vitamins. Although a simple fasting measurement of plasma
homocysteine can serve as a useful screening test, people with genetically inherited
hyperhomocysteinemia and those who are vitamin B6 deficient may have normal or
minimally elevated levels. A more sensitive method involves obtaining a second
measurement 4 hours after the patient consumes methionine; hyperhomocysteinemia is found
twice as often when this method is used.
In hyperhomocysteinemia, the endothelial lining of the vessel walls is denuded, which can
precipitate thrombus formation. Research findings do not support that taking folic acid,


vitamin B6,a nd vitamin B12 supplements is effective in decreasing the risk of recurrent
venous or arterial thromboembolism. Smoking causes low levels of vitamin B6 and B12 and
folate; thus, homocysteine levels rise.
Signs and symptoms
If the elevated levels are due to a deficiency in vitamins B-6 and B-12, or folate, a person
may experience symptoms such as:
 weakness
 dizziness
 sores on mouth or tongue
 tingling in the feet, legs, hands, or arms
 fatigue
 pale skin
Causes and Risk Factors
Vitamin B-6, B-12, and folate deficiencies are a common cause of higher homocysteine
levels in some people. But other factors include:
 family history
 genetics
 diet
 smoking
 alcohol
 diabetes
 rheumatoid arthritis
 Crohn’s disease
An article in the Journal of Geriatric Cardiology Trusted Source notes that homocysteine

levels may also increase as a person ages. Also, according to Food for the Brain, a not-for-
profit educational charity, males are more likely to have higher levels than females.
Complications


According to a 2017 meta-analysisTrusted Source, healthcare professionals associate high

homocysteine levels with all-cause mortality risks. For every 5 µmol/L increase in
homocysteine levels, there was a 33.6% increase of all-cause mortality risk.
Potential conditions associated with high homocysteine levels include:
 osteoporosis, which occurs when bones become weaker

 Parkinson’s disease, which is a disorder of the central nervous system
 dementia
 multiple sclerosis, which is an autoimmune disease that attacks the spinal cord and the
brain
 stroke
 epilepsy
 eclampsia, which is the onset of seizures caused due to high blood pressure
 aortic aneurysm, which occurs when an abnormal bulge develops in the aorta
 cardiovascular disease
 heart attack
 atherosclerosis, which is an arterial disease
 cancer
 end stage renal disease
 hypothyroidism, which is when the body is unable to produce thyroid hormones
Management
Nursing Management
 discuss the patient’s intake of foods with folic acid, vitamin B6, and B12
 increase foods rich in these vitamins such as fruits and vegetables as a portion of the
general population does not meet the Recommended Dietary Allowance (RDA) for these
nutrients.
Medical Mangement
 In patients who have homocystinuria with severe hyperhomocysteinemia, homocysteine-
lowering treatments with pyridoxine, folic acid, and hydroxocobalamin did reduce
cardiovascular risk
Antithrombin Deficiency


AT is a protein that inhibits thrombin and certain coagulation factors, and it may also play a
role in diminishing inflammation within the endothelium of blood vessels. AT deficiency is
most commonly a hereditary condition that can cause venous thrombosis, particularly when
the AT level is less than 60% of normal. The most common sites of thrombosis are the deep
veins of the leg and the mesentery. Recurrent thrombosis often occurs, particularly as the
patient ages. Patients tend to exhibit heparin resistance; thus, they may require greater
amounts of heparin to achieve adequate anticoagulation.
AT deficiency can also be acquired by four mechanisms:
 Accelerated consumption of AT (as in DIC)
 Reduced synthesis of AT( as in hepatic dysfunction)
 Increased excretion of AT (as in nephrotic syndrome)
 Medication induced (e.g., estrogens, L-asparaginase)
Protein C Deficiency
Protein C is a vitamin K-dependent enzyme synthesized in the liver; when activated, it
inhibits coagulation. When levels of protein C are deficient, the risk of thrombosis increases,
and thrombosis can often occur spontaneously.
People who are deficient in protein C are often without symptoms until their 20s; the risk of
having a thrombotic event then increases. Individuals with protein C deficiency are at a
higher risk for recurrent PE.
A rare but significant complication of anticoagulation management in patients with protein C
deficiency is warfarin-induced skin necrosis. This complication appears to result from
progressive thrombosis in the capillaries within the skin. The extent of the necrosis can be
extreme.
People also acquire the condition through non-genetic means, including:
 taking blood thinners, such as warfarin

 liver failure
 low intake of vitamin K
 surgical removal of the small intestine
 a course of antibiotics without adequate nutrition
 tumors throughout the body
 clotting disorders due to blood infections
 bacterial infections in young people
Risk factors


The greatest risk factors for the condition are having a parent with the condition or a family
history of abnormal blood clots.
Parents pass the mutation that causes protein C deficiency to their offspring. There is a 50
percent chance of inheriting the condition when one parent has it. More severe cases can
occur when individuals inherit mutated PROC genes from both parents.
The following factors increase the risk of blood clots:
 age
 surgery
 a lack of exercise
 pregnancy
 an accompanying blood-clotting disorder
Symptoms
The most severe cases of protein C deficiency occur shortly after birth. This is usually the
result of a blood-clotting condition called purpura fulminans.
Some people with very low levels of protein C might show no symptoms until puberty.
However, they are just as likely to have blood clots and blockages as a person who showed
symptoms earlier on.
Complications
Individuals may only discover they have protein C deficiency after blood clots and other
associated complications occur.
The complications of protein C deficiency can be severe and may lead to emergency
treatment. These include:
 Deep vein thrombosis

Deep vein thrombosis (DVT) is a serious condition that can develop among people with
even mild protein C deficiency. DVTs are blood clots that form below the surface of the
skin, usually in the arms and legs but also around the brain.DVTs can become life-
threatening when they move through the body and cause blockages in the lungs.
 Pulmonary embolism
This dangerous condition may develop after DVT. A pulmonary embolism blocks blood
flow to the lungs.


 Problems during pregnancy

Protein C deficiency raises the risk of clots for women during pregnancy and after labor.
The risk is higher after birth.
 Purpura fulminans
This is a life threatening condition occurring in infants with severe protein C deficiency.
Blood flow stops around these clots, causing cell death. The body uses up blood-clotting
proteins quickly, resulting in abnormal bleeding and discolored skin.
 Warfarin-induced necrosis
Blood clots cause cell death in the breasts, buttocks, thighs, or torso. Bleeding in these
areas turns them purple and blue and causes swelling, severe pain, and gangrene.
Treatment will involve immediately switching warfarin to a course of heparin, vitamin
K, and protein C concentrate.
Protein S Deficiency
Protein S is another natural anticoagulant normally produced by the liver. APC requires
protein S to inactivate certain clotting factors. When the level of protein S is deficient, this
inactivation process is diminished, and the risk of recurrent venous thrombosis early in life,
and also with recurrent PE.
Thrombosis most commonly occur in the axillary, mesenteric, and cerebral veins. Warfarin-
induced skin necrosis is possible. Acquired protein S deficiency can also occur. Pregnancy,
DIC, liver disease, nephritic syndrome, HIV infection, and the use of L-asparaginase have all
been associated with reduced protein S levels.
Activated Protein C Resistance and Factors V Leiden Muation

APC resistance is a common condition that can occur with other hypercoagulable states.
APC is an anticoagulant, and resistance to APC increases the risk of venous thrombosis. A
molecular defect in the factor V gene has been identified in most (90%) patients with APC
resistance.
This factor V Leiden mutation is the most common cause of inherited hypercoagulability in
Caucasians, but its incidence appears to be much lower in other ethnic groups. Factor V
Leiden mutation synergistically increases the risk of thrombosis in patients with other risk
factors (e.g., the use of oral contraceptives, hyperhomocysteinemia, increased age).
People who are homozygous for the factor V Leiden mutation are extremely high risk of
thrombosis and therefore need anticoagulation for life. In contrast, those who are
heterozygous for the mutation have a lower chance for developing thrombus. The duration of
anticoagulation is based upon the coexistence of other risk factors for thrombi formation.


Acquired Thrombophilias
Acquired thrombophilias are types of clotting disorders that do not have inherited/genetic
cause.
Etiology
Acquired thrombophilias result in inappropriate clot formation, typically caused by either an
excess in antibodies that cause clotting or an increase in clotting factors.
 Antiphospholipid
 Antibodies to phospholipids are common acquired causes of thrombophilia. These
antibodies reduce levels of annexin V, a protein that binds phospholipids and has
anticoagulant activity. The most common of these phospholipid antibodies are either
lupus or anticardiolipin antibodies, an antibody to B2-glycoprtein.
 Antiphospholipid syndrome is classified as primary or secondary, with a reaction
secondary to a pre-existing autoimmune disease- with systemic lupus erythematous being
the most common disease implicated
 Primary antiphospholipid syndrome is associated with certain infections or medications;
a genetic predisposition to this syndrome has been postulated but not yet proven
 Antiphospholipid antibodies are associated with repeated miscarriages and felt to be a
significant cause of stroke
 Most thrombotic events are venous, but arterial thrombosis can occur in up to one third of
the cases
 Thrombi typically occur in large vessels.
 Therapy varies based on type of syndrome, history of prior thrombosis, and location of
thrombosis; arterial thrombosis often necessitates adding low-dose aspirin to some form
of heparin
 Malignancy
Another common acquired cause of thrombophilia is cancer, particularly stomach,
pancreatic, lung, and ovarian cancers. The type of thrombosis that results is unusual. Rather
than DVT or PE, the thrombosis occurs in unusual sites, such as the portal, hepatic, or renal
vein or the inferior vena cava. Migratory superficial thrombophlebitis or nonbacterial
thrombotic endocarditis can also occur. LMWH appears to be a more effective anticoagulant
than warfarin in treating this patient population.
Management
Nursing Management
 Patient with thrombotic disorder should avoid activities that lead to circulatory stasis
 Exercise, especially ambulation, should be performed frequently throughout the day,
particularly during long trips by car or plane


 Patients with thrombotic disorder, particularly those with thrombophilia, should be

assessed for concurrent risk factors for thrombosis and should avoid them if possible.
 Frequent assessment should be performed for signs and symptoms of beginning thrombus
formation, particularly in the legs (DVT) and lungs (PE)
 Ambulation or range-of-motion exercises as well as the use of anti-embolism stockings
should be initiated promptly
Medical Management
Pharmacologic Therapy
 Anticoagulant agents are used in treatment or prevention of thrombosis. These agents,
particularly warfarin or unfractionated heparin, can cause bleeding, particularly if their
use is not carefully monitored.
 Heparin is a naturally occurring anticoagulant agent that enhances AT III and inhibits
platelet function. To prevent thrombosis, heparin is typically given as a subcutaneous
injection two or three times daily
 LMWH (e.g., dalteparin [Fragmin], enoxaparin[ Lovenox]) are special forms of heparin
that have more selective effects on coagulation
 Heparin-Induced Thrombocytopenia is a significant complication of heparin-based therapy.

HIT involves he formation of antibodies against the heparin-platelet complex.
 Women appear to be at a higher risk and young adults are at very low risk for developing the
disorder for the reasons that are not clear.
 A decline in platelet count is a hallmark sign that typically occurs after 5 to 10 days of
heparin therapy; therefore platelet count should be monitored in any patient beginning
heparin therapy.
 Treatment for HIT includes prompt cessation of heparin (including any heparin-coated
catheters) and initiation of an alternative means of coagulation
 Warfarin (Coumadin) is a vitamin K antagonist; therefore it interferes with the synthesis

of vitamin K-dependent clotting factors
 Thrombin and Factor Xa Inhibitors. Several oral anticoagulant agents have been
approved for use that are not Vitamin K antagonist (such as warfarin). Dabigatran
(Pradaxa) is a direct thrombin inhibitor, whereas rivaroxaban (Xarelto), apixiban
(Eliquis), and edoxaban (Savaysa) are Factor Xa inhibitors. These drugs are indicated for
prevention of DVT, PE or stroke in patients with atrial fibrillation.
 Aspirin
Discharge Plan
 Medications
- Instruct significant others to timely follow the ordered prescription and to strictly
monitor the right time, dose, frequency and route in giving the medications.
 Environment


- Encourage significant others to maintain the cleanliness of the house and surrounding.
- Refer patient to home care nurse, and assist in transition from hospital to home.
 Exercise/ Activity
- Encourage rest and probably a change in lifestyle (adequate, well-balanced diet and
elimination of alcohol).
- Advice to avoid doing strenuous activities.
 Treatment
- Instruct patient to comply medication.
- Encourage patient to go to follow up consultation on the prescribe date.
 Health Teaching
- Provide written instructions, teaching, support, and reinforcement to patient and family.
- Observe signs and symptoms that need reporting.
- Encourage patient and s/o to observe condition and if any unusual health problem to the
health care provider.
 Outpatient/Inpatient Referrals
- Advise to avoid activities that may cause scratches or bruises and use caution with skin
and mouth care
 Diet
- Encourage patient to have a healthy diet.
- Encourage patient to increase fluid and electrolyte intake.
 Spiritual
- Encourage patient to have a strong spiritual connection.

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Republic of the Philippines

JH CERILLES STATE COLLEGE

A Case Study on Bleeding Disorder and Acquired Coagulation Disorder

2nd Semester, A.Y. 2020-2021

School of Arts & Sciences

Bleeding disorders may also be caused by:

 a low red blood cell count

Signs and symptoms

 unexplained and easy bruising

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Common complications of bleeding disorders include:

 bleeding in the intestines

 Complete blood count (CBC)

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Increased platelet production is the primary mechanism of secondary, or reactive

Signs and Symptoms

 Easy or excessive bruising (purpura)

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

 Have certain types of cancer, aplastic anemia, or autoimmune diseases

 Prolonged bleeding, even from minor cuts

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Immune Thrombocytopenic Purpura

Signs and Symptoms

 Easy or excessive bruising (purpura)

 ITP is more common among young women.

ITP is an autoimmune disorder characterized by a destruction of normal platelets by an

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Antiplatelets antibodies develop in the blood and

Ingested and destroyed by the reticuloendothelial

Increase platelet production within the marrow

The antibodies may also induce cell death (via

Impaired platelet production

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Signs and symptoms

 Severe and/or mild bleeding

Two inherited bleeding disorders-hemophilia A and hemophilia B- are clinically

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Signs and symptoms

 Hemorrhage into various parts of the body

Hemophilia is an X-linked recessive genetic disease causing abnormal bleeding because of a

Hemophilia may be severe, moderate, or mild, depending on the degree of activation of

Excess of circulating thrombin Positive feedback

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

Blood vessel blockage by fibrin Organ failure

Diagnostic test results

 Cryoprecipitate (for hemophilia A) or lyophilized factor VIII or IX to increase clotting

NCM 116 – Medical-Surgical Nursing

School of Arts & Sciences

 Patients are encouraged to be self-sufficient and to maintain independence by preventing

Von Willebrand Disease (vWD)

There are three types of vWD:

Type 1: the most common, is characterized by decrease in structurally normal vWF.

Signs and symptoms