BLEEDING DISORDERS

Hemostasis
Refers to the stoppage of blood
Normal when it seals a blood
vessel to prevent blood
loss/hemorrhage
Abnormal when it causes
inappropriate blood clotting, or is
insufficient to stop blood flow from
the vasculature
Balance of pro-coagulants and anticoagulants that maintains:
Blood flow

Hemostasis
Vessel spasm
Mechanism
Transient -1min
TXA2 -prostaglandin f. Pltsvasoconstriction

Formation of the platelet plug

Plts in contact with vessel wall
Adhesion(vWF) and
aggregation(granules) Plts

Blood coagulation/insoluble fibrin clot

Activation of coagulation pathway
Fibrinogen to Fibrin mesh

Hemostasis
Clot retraction
Mechanism
Occurs 20-60min after a clot has
formed
Squeezes serum from the clot
Joining the edges of the broken
vessel

Clot dissolution
Fibrinolysis - begins shortly after its
formation
Allows blood flow to be reestablished
Balance between activators and
inhibitors

Coagulation
Liver is the major site of synthesis
Cascade
In severe liver disease all coagulation

factors are diminished except factor

VIII
Vitamin k dependent factors: II, VII, IX,
and X
Protein C and S are naturally occurring
anticoagulants which synthesis is also
vitamin k dependent
Warfarin blocks liver uptake of vitamin
K, decreasing the synthesis of vitamin

Coagulation
Artificially divided into extrinsic and
cascade
intrinsic pathways that converge into

the common pathway leading to

thrombin and fibrin generation
Extrinsic pathway assessed in the
laboratory by the Prothrombin time
(PT)
PT- sensitive to deficiencies of factors,
II,VII, V, and X, all these associated
with bleeding complications
PT- to monitor warfarin (coumadin)

Coagulation
PT-INR-international normalized ratio,
cascade
the degree of prolongation of PT by
warfarin depends on the strength of
the reagents used in the lab ,which
could vary among labs. INR created To
standardize the variations and allow
for global application of anticoagulant
recommendations
INR = patient PT/ mean control PT

Coagulation
PTT- partial thromboplastin time,
cascade
assess the intrinsic pathway
Sensitive to deficiencies of factor, II,
V,VIII,IX, X,XI
To monitor Heparin therapy
Heparin binds to antithrombin III, and
increases its ability to inactivate
thrombin, factor Xa and others

THROMBOCYTOPENI
A

Overview
Thrombocytopenia is the
medical term for a low blood
platelet count.
It is the most common bleeding
disorder.

Platelets
Platelets are produced in bone marrow
from Megakaryocytes
General platelet count ranges
150,000 450,000 microliter
Each platelet lives only about 10 days.
The risk of bleeding increase as
number of platelet decreases.

Classification of
thrombocytopenia
Mild:
100,000-150,000 microliter
Moderate: 50,000-100,000 microliter
Severe:
less than 50,000 microliter
The greatest risk is when platelet count
below 10,000 microliter

Causes
Common causes
Pregnancy
Gestational
thrombocytopenia

Drug-induced
Heparin, Quinidine,
Quinine, Sulfonamides

Viral infection
HIV, Infectious
mononucleosis,
Hepatitis

Hypersplenism due
to chronic liver
disease

Other causes
Myelodysplasia
Congenital
thrombocytopenia
Thrombotic
thrombocytopenic
purpura-hemolytic
uremic syndrome
Chronic disseinated
intravascular
coagulation

Causes of
Thrombocytopenia
Decreased
production
Bone marrow suppression or damage
Leukemia , Aplastic anemia
Viral infection [Include HIV infection]
Dengue fever
Chemo or radiation therapy
Congenital or acquired bone marrow aplasia
or hypoplasia

Causes of
Thrombocytopenia
Decreased production
Vitamin B12 or folic deficiency
Liver failure
Heavy alcohol consumption

Sepsis, systemic viral or bacterial

infection

Causes of
Thrombocytopenia
Increased destruction
Idiopathic (Immune) Thrombocytopenic
Purpura [ITP]
Alloimmune destructionPosttransfusion,
Post-transplantation
Disseminated Intravascular Coagulation
[DIC]
Thrombotic Thrombocytopenic Purpura
[TTP]
Hemolysis, elevated liver enzymes, low
platelets [ HELLP]

Increased
Systemic lupus erythematosus [SLE]
destruction

Dengue fever [cause shortened platelet

survival and immunological platelet
destruction]
Pregnancy relate Gestational
thrombocytopenia [Mild]

Medication induced
Heparin
Valproic acid
[Depakin]
Quinidine
Sulfonamide
antibiotic
MMR Vaccine
Clopidogrel
[Plavix]
Methotrexate
Carboplastin

Vancomycin
Famotidine
[Pepcine ,
Pepcidine ]
Mebeverine
[Colofac]
Metronidazole
Interferon

Clinical
manifestations
When platelet
Usually
asymptomatic
When platelet
count is below
20,000m3

- Petechiae occur
spontaneously
- Ecchymoses occur
at sites of minor
trauma
- Bleeding from
mucosal surfaces,

count is below
20,000m3
- Menorrhagia
- Excessive
bleeding after
procedures
(dental
extractions, minor
surgery, biopsies

Palpable purpura

Diagnostics
CBC Mild, Moderate, Severe
Blood smear
Pseudothrombocytopenia
Bone marrow test
Bone marrow aspiration ; done to
find out why your bone marrow isnt
making enough blood cells =>
remove a small amount fluid bone
marrow to check for abnormal cells
Bone marrow biopsy ; done right
after an aspiration => remove bone

Diagnostics
Bleeding time, prothrombin time (PT),
partial thromboplastin time (PTT) prolonged

Management:
Treat underlying cause
Medical

Platelet transfusion
Steroids or IV immunoglobulins

Management:
Institute bleeding precautions
Nursing

- Avoid use of plain razor, hard toothbrush

or floss, intramuscular injections,
tourniquets, rectal procedures or
suppositories
- Administer stool softeners

- Restrict activity and exercise when

platelet count is below 20,000m3

Management:
Monitor pad count/amount of
Nursing
saturation during menses
Administer blood products as ordered
Evaluate urine, stool and emesis for
gross and occult blood
Health teachings on:
- Avoid blowing nose
- Avoid use of aspirin and NSAIDs
- Demonstrate use of direct, steady
pressure at bleeding site
- Encourage routine follow-up for platelet

IDIOPATHIC
THROMBOCYTOPEN
C PURPURA

Overview
Idiopathic thrombocytopenic purpura
is ableeding disorderin which the
immune system destroys platelets,
which are necessary for normal blood
clotting.
Persons with the disease have too few
platelets in the blood.
ITP is sometimes called immune
thrombocytopenic purpura.

Overview
ITP occurs when certain immune
system cells produceantibodies
against platelets. Platelets help your
blood clot by clumping together to
plug small holes in damaged blood
vessels.
The antibodies attach to the platelets.
The spleen destroys the platelets that
carry the antibodies.

Overview
In children, the disease sometimes
follows a viral infection. In adults, it
is more often a chronic (long-term)
disease and can occur after a viral
infection, with use of certain drugs,
during pregnancy, or as part of an
immune disorder.
ITP affects women more frequently
than men, and is more common in
children than adults. The disease
affects boys and girls equally.

Clinical
Menorrhagia
manifestations

Bleeding into the skincauses a

characteristic skin rash that looks like
pinpoint red spots (petechial rash)
Easy bruising
Nosebleed or bleeding in the mouth

Diagnostics
CBC - shows a low number of platelets
- Below 20,000m3 (acute)
- 30,000-70,000m3 (chronic)

Blood clotting tests (PTTandPT) normal

Bleeding time - prolonged
Platelet associated antibodies- may
be detected
Bone marrow biopsy appears normal
or may show increased number of
megakaryocytes

Management:
Anti-inflammatory steroid medicine
Medical
prednisone
Splenectomy - will increase the
platelet count in about half of all
patients
Danazol (Danocrine) - taken orally
High-dose gamma globulin
Drugs that suppress the immune
system
Filtering antibodies out of the blood
stream

Management:
Institute bleeding precautions
Nursing

- Avoid use of plain razor, hard toothbrush

or floss, intramuscular injections,
tourniquets, rectal procedures or
suppositories
- Administer stool softeners

- Restrict activity and exercise when

platelet count is below 20,000m3

Management:
Monitor pad count/amount of
Nursing
saturation during menses
Administer blood products as ordered
Evaluate urine, stool and emesis for
gross and occult blood
Health teachings on:
- Avoid blowing nose
- Avoid use of aspirin and NSAIDs
- Demonstrate use of direct, steady
pressure at bleeding site
- Encourage routine follow-up for platelet

DISSEMINATED
INTRAVASCULAR
COAGULATION

Overview
Disseminated intravascular
coagulation (DIC) is a serious disorder
in which the proteins that control
blood clotting become abnormally
active.
Normally when you are injured, certain
proteins in the blood become
activated and travel to the injury site
to help stop bleeding. However, in
persons with DIC, these proteins
become abnormally active. This often
occurs due to inflammation,

Overview
Smallblood clotsform in the blood
vessels. Some of these clots can clog
up the vessels and cut off blood
supply to various organs such as the
liver, brain, or kidney. These organs
will then be damaged and may stop
functioning.
Over time, the clotting proteins are
consumed or "used up. When this
happens, the person is then at risk for
serious bleeding, even from a minor
injury or without injury. This process

Risk factors
Blood transfusion reaction
Cancer - especially certain types of
leukemia
Infection in the blood by bacteria or
fungus
Liver disease
Pregnancy complications abruptio
placenta and retained placenta
Recent surgery or anesthesia
Sepsis
Severe tissue injury burns and head

Clinical
Signs of abnormal clotting
manifestations
- Coolness and mottling of extremities
-

Acrocyanosis
Dyspnea, adventitious breath sounds
Altered mental status
Acute renal failure
Pain

Clinical
Signs of abnormal bleeding
manifestations
- Oozing, bleeding from sites of procedures
- Internal bleeding leading to changes in
vital organ function, altered vital signs

Diagnostics
Complete blood countwith blood
smear examination
Fibrin degradation products
increased
Partial thromboplastin time (PTT)
prolonged
Platelet count diminshed
Prothrombin time(PT) prolonged
Serum fibrinogen decreased
Antithrombin III decreased

Management:
The goal is to determine and treat the
Medical
cause of DIC.
Replacement therapy
- Fresh frozen plasma
- Platelet transfusion
- Cryoprecipitate

Supportive measures
- Fluid replacement
- Oxygenation replacement and
monitoring
- Maintenance of blood pressure and renal

Management:
Institute bleeding precautions
Nursing

- Avoid use of plain razor, hard toothbrush

or floss, intramuscular injections,
tourniquets, rectal procedures or
suppositories
- Administer stool softeners

- Restrict activity and exercise when

platelet count is below 20,000m3

Management:
Monitor pad count/amount of
Nursing
saturation during menses
Administer blood products as ordered
Avoid dislodging clots. Apply pressure
to sites of bleeding for at least 20
minutes
Maintain bed rest during bleeding
episodes
If internal bleeding is suspected,
assess bowel sounds and abdominal
girth

Management:
Promoting tissue perfusion
Nursing

- Keep warm
- Avoid vasoconstrictive agents
- Change position frequently and perform
ROM
- Monitor ECG and laboratory tests for
dysfunction of vital organs caused by
ischemia
- Monitor for signs of vascular occlusion
Eyes visual deficits
Bone bone pain
Extremities cold, mottling, numbness

Complications
Bleeding
Lack of blood flow to extrimeties and
vital organs
Stroke

HEMOPHILIA

Overview
Hemophilia is a bleeding disorder that
slows down the blood clotting process.
People who have Hemophilia often
have longer bleeding after some sort
of contact to injury.
People who have severe Hemophilia
start to have spontaneous bleeding in
the joints and muscles all around your
body.
Hemophilia is more common in males
than females.

Types of Hemophilia
Hemophilia A:
Also known as classic hemophilia or Factor VIII
Deficiency
People with this type of hemophilia have low levels of a
blood clotting factor called figure 8 (FVIII)
Hemophilia B:
Also known as Christmas disease or Factor IX
Deficiency
People with this type of hemophilia have low levels of a
blood clotting factor called figure 9 (FIX)
-The two different types of hemophilia are caused by
permanent gene changes (mutations). Mutations in
the FVIII gene cause Hemophilia A. Mutations in the FIX
gene cause Hemophilia B.

Hemophilia A
Also known as classic hemophilia
People with this type of hemophilia have
low levels of a blood clotting factor called
figure 8 (FVIII)
-Severe Hemophilia A: Spontaneous joint
or deep muscle bleeding. Usually diagnosed
within first two years of life.
-Moderate Hemophilia A: spontaneous
bleeding, delayed oozing after minor injury,
and usually diagnosed before they are 5 to 6
years old.
- Mild Hemophilia A: Do NOT have
spontaneous bleeding but unusual bleeding
occurs with surgery and tooth extractions.

Hemophili
aA

Hemophilia B
Also known as Christmas disease
People with this type of hemophilia have
low levels of a blood clotting factor called
figure 9 (FIX)
-Severe Hemophilia B: Spontaneous joint or
deep muscle bleeding is the most frequent
symptom. People are usually diagnosed in the
first 2 years of life.
-Moderate Hemophilia B: spontaneous
bleeding, delayed oozing after minor injury,
and usually diagnosed before they are 5 to 6
years old
-Mild Hemophilia B: Do NOT have
spontaneous bleeding but unusual bleeding

Which type is more

Hemophilia A is more common than
common?
Hemophilia B. One in 5000-10000
males around the world have
Hemophilia A. One in 20,00034,500 males around the world
have Hemophilia B.

Clinical manifestations
nose bleeds that dont stop
painful/ swollen joints
excessive bleeding from having a tooth pulled
unusual bruises
blood in urine

Diagnostics
Hemophilia A&B are diagnosed by measuring factor
clotting activity. Individuals who have Hemophilia
A have low factor VIII clotting activity. Individuals
who have hemophilia B have low factor IX clotting
activity. Genetic testing is also available for the
factor VIII gene and the factor IX gene.
Genetic testing of the FVIII gene finds a
disease-causing mutation in up to 98 percent
of individuals who have hemophilia A.
Genetic testing of the FIX gene finds diseasecausing mutations in more than 99 percent of
individuals who have hemophilia B.
Used to identify women who are carriers of a
type FVIII or FIX gene mutation, diagnose
hemophilia in a fetus during a pregnancy, and

Is Hemophilia inherited?
Hemophilia A and hemophilia B are inherited in an Xlinked recessive pattern. The genes associated with
these conditions are located on the X chromosome, which
is one of the two sex chromosomes. In males (who
have only one X chromosome), one changed copy of
the gene in each cell is sufficient to cause the condition.
In females (who have two X chromosomes), a
mutation would have to occur in both copies of the gene
to cause the disorder. Because it is unlikely that females
will have two changed copies of this gene, it is very rare
for females to have hemophilia. A characteristic of Xlinked inheritance is that fathers cannot pass X-linked
traits to their sons. In X-linked recessive inheritance, a
female with one changed copy of the gene in each cell is

Right now, there is no cure for

Hemophilia but, there are some
treatments being used depending
on the` severity of hemophilia

Management:
Institute bleeding precautions
Nursing

- Avoid use of plain razor, hard toothbrush

or floss, intramuscular injections,
tourniquets, rectal procedures or
suppositories
- Administer stool softeners

- Restrict activity and exercise when

platelet count is below 20,000m3

Management:
Monitor pad count/amount of
Nursing
saturation during menses
Administer blood products as ordered
Evaluate urine, stool and emesis for
gross and occult blood
Health teachings on:
- Avoid blowing nose
- Avoid use of aspirin and NSAIDs
- Demonstrate use of direct, steady
pressure at bleeding site
- Encourage routine follow-up for platelet

Preventions
When people that have a serious case of
hemophilia they have the option of getting a
series of shots. They are known as clotting
factor replacement therapy.

END