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Hematologic Problems

N323 Module B part I

ANEMIA
1.) Anemia is reduction in either RBCs, amount of hemoglobin,
or hematocrit (% of packed RBC per deciliter of blood)
Anemia is a symptom of an underlying disease.
Causes and types vary:
1.)dietary problemsdeficiency in components necessary
to make RBCiron, vitamin B12 (cyanocobalamin), folic
acid, or intrinsic factor

2.)genetic disorders

3.)bone marrow disease

4.)excessive bleeding
5.)Immune reactions
6.)Changes in blood chemistry
7.)Toxins in the blood
**Gastrointestinal bleeding common cause of anemia in
adults**
N323 Module B part I

Chronic vs. Acute Anemia


Chronic anemias develop gradually,
more subtle symptoms-- lethargy, pallor,
and anorexia (gastritis, hemorrhoids, menstrual
flow)

Acute anemias do not allow the body


sufficient time to make physiologic
adjustments -- patients symptomatic with
shortness of breath, extreme fatigue, and
cardiac discomfort (trauma, blood vessel rupture)
N323 Module B part I

Hematologic Problems
Anemias
Results in: reduction in oxygen transport due to
decrease in hemoglobin production, a decrease
in erythrocytes, or a combination of these
factors.
Reduced oxygen leads to less energy in all cells,
reduced cell metabolism and reproduction.
Compensation mechanisms include tachycardia
and peripheral vasoconstriction

N323 Module B part I

Hematologic Problems

Anemias
General signs of anemia: fatigue, pallor,
dyspnea, and tachycardia
Severe anemia may lead to angina if
oxygen supply to the heart is insufficient
Chronic severe anemia may cause CHF
Other affects may include hair and skin
changes
Cultural considerations:
N323 Module B part I

Key Features of Anemia


Integumentary manifestations
Respiratory
Pallor, of ears, nail beds, palmar
Manifestations
creases, conjunctiva, and around
mouth
Dyspnea on exertion
cool to touch
Decreased oxygen
intolerance of cold temperatures
Nails become brittle, overtime
saturation levels
become concave and fingers are
club like in appearance.
Neurologic Manifestations
Cardiovascular Manifestations
Increased somnolence
Tachycardia, murmurs, gallops
when anemia severe orthostatic
and fatigue
hypotension
Headache

N323 Module B part I

Diagnostic Assessment
Tests of cell number and function:
Complete blood count
Reticulocyte count
Hemoglobin electrophoresis
Serum ferritin
transferrin
total iron-binding capacity

N323 Module B part I

LABORATORY PROFILE
Test
Significance of abnormal finding
Red blood cell count
Decreased indicate possible
anemia/hemorrhage
Hemoglobin/Hematocrit
Increased indicate possible chronic
hypoxia, or polycythemia vera
Mean cell hemoglobin (MCV)
Increased levels indicate macrocytic cells,
possible anemia. Decreased levels
indicate microcytic cells,
possible iron deficiency anemia
Reticulocyte count
helpful in determining bone marrow function
(immature RBC) **Increased levels indicate
chronic blood lossdesireable in anemic client
or after hemorrhage.

N323 Module B part I

Hemoglobin electrophoresis
as

detects abnormal forms of hemoglobin, such

hemoglobin S in sickle cell disease,


Prothrombin time /INR
assesses extrinsic clotting cascade,
reflects how much clotting
factors II, V, VII, X is functioning.
Increased=deficient in clotting factor
cascade.
Decreased=vitamin K excess.
(monitors Coumadin tx) 25-38 sec
PTT aPartial thromboplastin time assesses the intrinsic clotting cascade,
factors VIII, IX, XI, XII.
Prolonged w/hemophilia or
disseminated
intravascular coagulation (DIC).
(monitors Heparin)
Level maintained 1.5 to 2.5 times their
baseline values
N323 Module B part I

International normalized ration (INR) is the


recommended laboratory measurement system for
monitoring the effect of oral coagulation therapy.
Most patients on oral anticoagulants, the
therapeutic range of the INR is 2-3

Dehydration: When dehydration is present


hemoconcentration occurs resulting in :
increased hemoglobin, hematocrit, serum
osmolarity, glucose, protein, blood urea nitrogen,
and various electrolytes.
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3.) Hypoproliferative anemia. Hypoproliferative anemia can


be subdivided into three classes based upon the size of the
RBCs. The cells may be larger than normal (macrocytic),
normal (normocytic), or smaller than normal (microcytic).
Macrocytic anemia. Macrocytic anemia can be due to
several causes. The first is a deficiency in vitamin
B12 or folate, both important ingredients in RBC
production.
Microcytic anemia. Microcytic anemia is due to
abnormalities in the production of the essential RBC
protein, hemoglobin. This is often to due to underlying
disease, such as thalassemia, iron deficiency anemia
Normocytic anemia. Normocytic anemia may be due to
chronic disease including malnutrition or mixed anemia
(combined macrocytic and microcytic anemia).
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Assessment Hematologic
Demographic data (age,
gender)
Family history and genetic risk
Personal history (use of ASA,
NSAIDS, antibiotic useprolonged can lead to bone
marrow suppression)
Diet history
Socioeconomic status
Current health status
(Gordons Hematologic assessment
activity-exercise pattern, and nutritionmetabolic pattern pg 879)

Skin
Head and neck
Respiratory
Cardiovascular
Renal and urinary
Musculoskeletal
Abdominal
Central nervous
system
Psychosocial

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Anemia
Clinical Manifestations
Severe = Hb <6 g/dl

Mild = Hb 10 to 14 g/dl
Involve multiple body
May exist without symptoms
systems
Moderate = Hb 6 to 10 g/dl
Integument
Increased cardiopulmonary symptoms Eyes
Experienced at rest or during activity
Mouth

N323 Module B part I

Cardiovascular
Pulmonary
Neurologic
Gastrointestinal (GI)
Musculoskeletal

13

ANEMIAS RESULTING FROM DECREASED


PRODUCTION OF RBCs
B.

Iron Deficiency Anemia (RBC small, microcytic)


Mild to severe cases
A sign of underlying problem, important find cause
Occurs all age groups
Insufficient iron impedes synthesis of hemoglobin,
reducing the amounts of oxygen transported in
blood
Results in microcytic (small cell) hypochromic (less
color) erythrocytes due to a low concentration of
hemoglobin in each cell.

N323 Module B part I

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Iron-Deficiency Anemia
One of the most common chronic
hematologic disorders
Iron is present in all RBCs as heme in
hemoglobin and in a stored form
Heme accounts for two thirds of the
bodys iron

N323 Module B part I

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Iron Deficiency Anemia (RBC small, microcytic)


Causes depleted iron stores (decreased ferritin),

results in a decreased supply of iron for the


manufacture of hemoglobin in RBCs
Iron stores 2/3 hemoglobin, 1/3rd in the bone marrow,
spleen, liver, and muscle
Many causes:
Lack of dietary intake of iron containing foods
(adolescent growth spurt, pregnancy need higher
amount of iron)
Slow, chronic blood loss from bleeding ulcer,
hemorrhoids, cancer, or excessive menstrual flow
syndromes of GI malabsorption
Occurs at any age, frequent women, older adults,
people poor diets
N323 Module B part I

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Iron Deficiency Anemia (RBC small, microcytic)


Signs and Symptoms:
Mild anemia frequently asymptomatic
Pallor skin and mucous membranes
Fatigue, lethargy
Cold intolerance (caused by decreased cell
metabolism)
Irritability ( CNS hypoxia)
Degenerative changes including brittle hair, spoon
shaped (concave) ridged nails,
Females: menstrual irregularities
Severe anemia: tachycardia, palpitations, dyspnea,
syncope, as well as delayed healing.
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Iron Deficiency Anemia (RBC small, microcytic)


Diagnostic Tests:
Hemoglobin, hematocrit (low)
Mean corpuscular volume (MCV), mean
corpuscular hemoglobin (MCH)
Serum ferritin less than12 g/L
Serum iron
Erythrocytes appear hypochromic and
microcytic on microscopic examination

N323 Module B part I

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Iron Deficiency Anemia (RBC small, microcytic)

Management

Find underlying cause


increase oral intake of iron, iron supplements
(take w/food to reduce gastric irritation and
nausea)
Oral treatment in 4 weeks raises Hgb about
2/g/dl
Liquid form of iron stains teeth and dentures
use straw
IMuse Z-track method, ventro-gluteal

injection

do not massage site


N323 Module B part I

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Iron-Deficiency Anemia
Drug Therapy

Oral iron
Vitamin C aids in iron absorption
Factors to consider (contd)
Best absorbed as ferrous sulfate in an acidic
environment
Liquid iron should be diluted and ingested
through a straw to prevent staining of teeth
Side effects
Heartburn, constipation, black stools,
diarrhea

N323 Module B part I

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Iron-Deficiency Anemia
Nursing Management

At-risk groups

Premenopausal women
Pregnant women
Persons from low socioeconomic
backgrounds
Older adults
Individuals experiencing blood loss

N323 Module B part I

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Iron-Deficiency Anemia
Nursing Management

Diet teaching
Supplemental iron
Discuss diagnostic studies
Emphasize compliance
Iron therapy for 2 to 3 months after
the hemoglobin levels return to normal

N323 Module B part I

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Common Food sources of Iron, Vitamin


B12, and Folic Acid
Iron
liver , organ meats
whole wheat breads/cereals
veges
Leafy green leafy veges, carrots
yeast
Egg yolks, raisins

Vitamin B12
liver, organ meats, nuts
dried beans, green leafy
citrus fruit, brewers

Folic Acid
Liver, organ meats, eggs, cabbage
Brocolli, brussel sprouts
N323 Module B part I

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Megaloblastic Anemias
Group of D/O caused by DNA synthesis and
characterized by large RBCs (abnormal =
hemolysis)
Common: Vitamin B12 Cobalamin deficiency
anemia
Pernicious anemia lack of secretion of intrinsic
factor (needs acidic environment) in GI tract which
is necessary for absorption of Vitamin B12
Folic acid anemia
Other causes: certain drugs
N323 Module B part I

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Vitamin B12 Deficiency Anemia (Cobalamin) &


Pernicious anemia) (megaloblastic, macrocytic)
Characterized by very large nuclueated,
immature erythrocyte
Vitamin B12 is required for RBC maturation
Causes: deficiency in B12 (cyanocobalamin)
results in inhibiting folic acid acid transport into
the cell =RBC maturity does not can occur. (all
cell division requires adequate amount of folic
acid to make DNA)

N323 Module B part I

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Vitamin B12 (Cobalamin) Deficiency


Anemia & Pernicious anemia)
(megaloblastic, macrocytic)

Results from poor intake of B12 related to


vegetarian diets, or diets lacking in dairy
products,
OR poor absorption of B12 in conditions
such as small bowel resection, tapeworm,
or overgrowth of intestinal bacteria

N323 Module B part I

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Vitamin B12 Deficiency Anemia &


Pernicious anemia (megaloblastic,
macrocytic)
Key Features of Vitamin B12 Anemia:
severe pallor

slight jaundice
Smooth, shiny, beefy tongue
weight loss
paresthesia of hands and feet (numbness and
tingling), difficulty w/gait related lack of B12
needed for normal nerve function (impaired
conduction of nerve impulses=demyelination)
peripheral nerves and eventually spinal cord)
N323 Module B part I

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Vitamin B12 Deficiency Anemia & Pernicious


anemia) (megaloblastic, macrocytic)
Diagnostic tests:
Erythrocytes appear macrocytic or megaloblastic
Serum vitamin B12 low
Shilling test is used to measure absorption of vitamin
B12 after oral administration.
Treatment: prophylaxis oral supplements for
pregnant women and vegetarians.
Vitamin B12 injection daily X2 weeks initially, then
weekly until hemoglobin normal, then monthly for
maintenance for life

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Folic acid deficiency (also cause


megaloblastic anemia)

Required for DNA synthesis L/T RBC formation &


maturation

Manifestations: similar to Vitamin B12 anemia, except


nervous system function remains normal. Disease
develops slowly.
Causes: common include poor nutrition,(r/t chronic
alcoholism), malabsorption problems (Crohns disease),
and drugs (oral contraceptives, anticonvulsants), HD
Collaborative management:
Identify high risk groups (elderly, debilitated, alcoholic,
those susceptible to malnutrition, and those who require
more (pregnancy)
Scheduled folic acid replacement therapy
N323 Module B part I

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Thalassemia (Cooleys anemia)


Etiology

An autosomal recessive genetic disorder of inadequate


production of normal hemoglobin
Normal hemoglobin, also called hemoglobin A, has four
protein chainstwo alpha globin and two beta globin.
In Thalassemia some of these proteins are missing L/T
abnormal hemoglobin
Common in ethnic groups near the Mediterranean
Sea and equatorial regions of Asia and Africa

N323 Module B part I

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Thalassemia
Etiology

Hemolysis also occurs


Problem with globulin protein
Abnormal Hb synthesis

One thalassemic gene (mild form)


Thalassemia minor (thalassemic trait)

Two thalassemic genes (severe form)


Thalassemia major

N323 Module B part I

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Thalassemia

Clinical Manifestations
Thalassemia major

Thalassemia minor

Asymptomatic frequently
Moderate anemia
Splenomegaly
Mild jaundice
Thalassemia major

Life-threatening
Physical and mental
growth often retarded
Pale
Symptoms develop in
childhood

Splenomegaly
Hepatomegaly
Jaundice from
hemolysis
Chronic bone marrow
hyperplasia

N323 Module B part I

Expansion of bone
marrow space

32

Thalassemia
Collaborative Care

No specific drug or
diet is effective in
treating thalassemia
Thalassemia minor
Body adapts to
decreased Hb

Thalassemia major

Prevention
Genetic counseling
with families with
known history of
Thalassemia
Prenatal checkup

Blood transfusions
with IV deferoxamine

(binds with iron, rids through


kidneys, TX, hematochromotosis)

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Glucose-6-Phosphate Dehydrogenase (G6PD)


Deficiency Anemia
Most common type of congenital hemolytic anemia
where this enzyme is lacking in the RBC
Enzyme lacking in the critical step of RBC energy
production
When exposed to certain drugs or toxins the aging RBC
easily break
Client usually asymptomatic until anemia or severe
infection develops
Acute phase: anemia and jaundice
Hemolytic reaction is limited because only older RBCs
containing less G6PD, are destroyed
N323 Module B part I

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Glucose-6-Phosphate Dehydrogenase (G6PD)


Deficiency Anemia
Collaborative management:
Hydration during episode of hemolysis to
prevent acute tubular necrosis
Identify and remove toxins or drug
Screening for this deficiency necessary before
donating blood
Osmotic diuretic such as mannitol (Osmitrol)
Blood transfusion to correct anemia

N323 Module B part I

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Aplastic Anemia (macrocytic)


Cause: deficiency of circulation RBC due to failure of bone
marrow to produce these RBC cells, may occur alone or with
Leukopenia (decreased WBC) and
thrombocytopenia (decreased platelets). When ALL three
occur together it is called Pancytopenia
Cause:
1.) Congenital in origin chromosomal abnormality
2.) Acquired--Long term exposure to toxic agents, ionizing
radiation or infection, (viral, bacterial), medications,
antiseizure, antimicrobials) may cause Aplastic anemia.
3.) 70% of acquired is idiopathic (unknown cause)

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Signs and Symptoms:


Onset insidius:
manifestations include those of anemia
Those of leukopenia (recurrent multiple infections)
Those related to thrombocytopenia (petechia,
tendency to bleed excessively,
especially in the mouth.

N323 Module B part I

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Aplastic Anemia (macrocytic)


Diagnosis: definitive bone marrow aspiration red
bone marrow is replaced by fatty red bone marrow
Treatment: blood transfusions, immunosuppressive
therapy (antilymphocyte globulin (ALG), cyclosporine
(Sandiuumne), prednisone, cyclophosphamide
(Cytoxan) can bring about partial or complete
remissions.
Splenectomy on clients with enlarged spleen that is
either destroying normal RBCs or suppressing their
development
Bone marrow transplant.

N323 Module B part I

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Anemias of Chronic Disease


Chronic inflammatory
Autoimmune
Infectious
Malignant diseases
Examples:
Renal disease (decreased ereythropoietin)
Myelosuppression
Medicationchemotherapy
Radiation
N323 Module B part I

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Acute Blood Loss


and
Chronic Blood Loss

N323 Module B part I

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Acute Blood Loss

20% volume loss may see signs or symptoms with increased


activity, slight postural hypotension Concerns
Result of sudden hemorrhage
Hypovolemic shock
Trauma
Reduced plasma
Complications of surgery
volume
Disruption vascular integrity
Diminished O2
because fewer RBCs
available

N323 Module B part I

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Acute Blood Loss


Clinical Manifestations

Cause

Bodys attempt to maintain an adequate blood volume


and O2

Pain
Internal hemorrhage
Tissue distention, organ displacement, nerve compression

Pain (cont'd)

Retroperitoneal bleeding
Numbness
Pain in the lower extremities

Shock is the major complication


N323 Module B part I

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Acute Blood Loss


Diagnostic Studies
Laboratory data does not adequately
assess RBC problems for 2 to 3 days
Collaborative Care
Replacing blood volume to prevent shock
Identifying the source of the hemorrhage
Stopping blood loss
Correcting RBC loss
N323 Module B part I

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Acute Blood Loss


Nursing Management

May be impossible to prevent if caused by


trauma
Postoperative patients
Monitor blood loss

No need for long-term treatment

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Chronic Blood Loss


Reduced iron stores

Bleeding ulcer
Hemorrhoids
Menstrual and postmenopausal blood loss

Management

Identify source
Stop bleeding
Possible use of supplemental iron

N323 Module B part I

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Hemolytic Anemia
Destruction or hemolysis
of RBCs at a rate that
exceeds production
Third major cause of
anemia
Intrinsic hemolytic
anemia
Abnormal hemoglobin

Extrinsic hemolytic
anemia
Acquired (mechanical injury
heart bypass, toxins)

Sites of hemolysis
Intravascular
Extravascular

(sickle cell)

Enzyme deficiencies
RBC membrane
abnormalities
N323 Module B part I

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Hemolytic Anemia
Jaundice
Destroyed RBCs
cause increased
bilirubin

Enlarged spleen and


liver

Accumulation of
hemoglobin
molecules can
obstruct renal tubules
Tubular necrosis

Hyperactive with
macrophage
phagocytosis of the
defective RBCs
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2.) Hemolytic Anemias


resulting from
increased destruction
(hemolysis) of RBCs
a.) Sickle cell disease

b.) Glucose-6Phosphate
Dehydrogenase
Deficiency anemia

c.) Thrombotic

Thrombocytopenia
Purpura (TTP) is a
rare autoimmune
reaction in blood
vessels disorder in
which platelets clump
together abnormally in
the capillaries and few
remain in circulation.

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Sickle Cell Disease (can cause anemia resulting from


increased destruction of RBCs)
1.) Cause genetic defect causing a formation of abnormal
hemoglobin chains (HbS) resulting in chronic anemia, pain,
disability, organ damage, increased risk for infection, and
early death

Sickle cell disease occurs in 1 in 350-500 African Americans


Sickle cell disease state vs. sickle cell trait, variation of
severity
Sickle cell trait less than 40% of total hemoglobin HbS
Sickle cell disease 80-100% of total hemoglobin contains
may have abnormality of hemoglobin S (HbS)

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2.) Insufficient oxygen


= cells to assume a
sickle shape,
cells become rigid and
clumped together,
obstructing small blood
vessels or the capillary
blood flow, cause venous
stasis, anemia, pain,
enlarged spleen
other manifestations in
the respiratory,
genitourinary,
cardiovascular,
musculoskeletal and
integumentary systems.

Obstruction of the small


blood vessels = repeated
multiple infarctions or
areas of tissue necrosis
throughout the body
Deoxygenation of
hemoglobin in peripheral
circulation = sickle cell
crisis

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Clinical Manifestations

Cardiovascular changes
Skin changes
Abdominal changes
Musculoskeletal changes
Central nervous system changes

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Diagnosis

Hemoglobin S (HbS) electrophoresis


Diagnosis confirmed by the presence of
sickled cells in peripheral blood
Hematocrit low
Reticulocyte count high (chronic anemia)
Mean corposcular hemoglobin
concentration (MCHC) high
bilirubin levels are high
WBCs elevated (due to chronic
inflammation and hypoxia)
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Interventions
include:

Protection of the
client from infection
in sickle cell crisis
Drug therapy
-pain meds, folic
acid replacement -Hydroxyurea (Droxia)

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Care of the Client in


Sickle Cell Crisis
Administer oxygen
Pain
Drug therapy: 48
hours of intravenous
analgesics
(opiodsMorphine or
Dilaudid) IV or PCA

Hydrate with IVF,


encourage PO fluids
4liters/day

Administer blood
transfusion if required
Remove constrictive
clothing
Encourage client to keep
extremities extended to
promote venous return
Check circulation of
extremities
Complementary and
alternative therapies

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Potential for Multiple Organ


Dysfunction
Interventions include:
- pain management
Hydration IV, PO
Oxygen therapy
Transfusion therapy
Hydroxyurea (Droxia)-(stimulate HbF, fetal hemoglobin

production, reducing HbS, LT use S/E bone marrow suppression, causes


birth defectsfemales should use 2 methods of birth control)

Complementary and Alternative


Therapies
(TENS, acupuncture)
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Acquired Hemolytic Anemia

Three extrinsic categories


1. Physical factors
Physical destruction of RBCs
results from extreme
force on the cells
Hemodialysis,
extracorporeal
circulation,
prosthetic heart
valves, angiopathic
disease (any disease of

2. Immune reactions

vessels)

Antigenantibody
reactions destroy
RBCs
Isoimmune reactions
Antibodies develop
against antigens;
blood transfusions

Autoimmune
reactions
Develop antibodies
against their own
RBCs

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Acquired Hemolytic Anemia

Three extrinsic categories


3. Infectious agents and toxins

Foster hemolysis in four ways

Invading RBCs and destroying contents


Releasing hemolysis substances
Generating an antigenantibody reaction
Contributing to splenomegaly

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Hemochromatosis
Primary hemochromatosis is an
inherited disorder characterized by
excessive iron accumulation due to
increaxed intestinal iron absorption
causing tissue damage.
Symptoms do not develop until organ
damage, often irreversible, develops.

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Symptoms include fatigue,


hepatomegaly, bronze skin
pigmentation, loss of libido,
arthalgias, and manifestations of
cirrhosis, diabetes, or
cardiomyopathy.
Diagnosis is based on serum iron
studies and gene assay.
Treatment serial phlebotomies
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Anemia
Nursing Implementation

Dietary and lifestyle changes Gerontologic


Considerations
Blood or blood product transfusions
Common in older adults
Drug therapy
Chronic disease
Oxygen therapy
Nutritional deficiencies
Patient teaching
Signs and symptoms may
Nutrition intake
go unrecognized or
Compliance with drug therapy
mistaken for normal aging
changes

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Polycythemia
Production and
Polcycythemia
Vera=(PV) is a rare
presence of increased
disease with a
RBCs
sustained increase in
2 types:
blood cells (primarily

Primary polycythemia =
red blood cells)
Polycythemia Vera
produced by the bone
marrow
Secondary Polycythemia =
a.) hypoxia driven = high
With unknown origin,
altitude, cardiopulmonary
chromosomal defect in
disease, defection O2
pluripotent stem cells
transport
b.)Hypoxia independent=
renal cysts or tumors

It is a cancer of the RBCs with 3


major hallmarks:
1.) Massive production of red blood
cells
2.) Excessive leukocyte production
3.) Excessive production of platelets
Hgb levels to 18 g/dl
Hct of 55% or >
RBC count of 6 mil/mm3
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Polycythemia Vera
Due to hyperviscous
(thicker than normal
blood) the following may
occur:
Key features:
Clients facial skin and
mucous membranes have
a dark, flushed
(plethoric) appearance
Distention of superficial
veins
Weight loss
Intense itching
Hypertension

Fatigue, enlarged
hemorrhoids
Swollen painful joints
Enlarged firm spleen
Infarctions of the heart
(chest pain, heart
failure), kidneys
Strokes
Bleeding tendency

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Polycythemia Vera
Diagnostic Tests
Blood cell counts and
hematocrit markedly
elevated
Hyperuricemia due to
high cell destruction
Bone marrow
hypercellular
Hgb levels to 18 g/dl
Hct of 55% or >
RBC count of 6 mil/mm3
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Polycythemia vera (PV)

Collaborative
management:

Phlebotomy (treatment)
blood drawing
Increase hydration
Anticoagulants are part
of therapy to prevent
clot formation

Chemotherapy to
suppress bone marrow
activity
Radiation therapy
Bone marrow
transplantation
Significant number of
individuals with PV go on
to develop acute
leukemia

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Polycythemia vera (PV)

Client education guide


Drink at least 3 L day
Avoid tight or
constrictive clothing,
especially garters or
girdles
Wear gloves when
outdoors in temperature
lower than 50 degrees
Contact physician first
sign of infection
Use soft-bristled
toothbrush
Do not floss teeth

Take anticoagulants as
prescribed
Wear support hose
while awake and up
Elevate feet when you
are seated
Exercise slowly and only
on the advice of your
physician
Stop activity at the
first sign of chest pain
Use electric shaver

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White Blood Cell Disorder


Leukemia

A group of malignant disorders affecting the


white blood cells and infiltrates tissues of:
--Bone marrow
--Lymph system
--Spleen
--Brain, & other organs
Leukemia cells ultimately occupy bone marrow and
circulate through the blood stream.

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Leukemia
Occurs in all age groups
Accumulation of dysfunctional cells due to loss
of regulation in cell division
Fatal if untreated
Often thought of as a childhood disease
The number of adults affected is 10 times that
of children
No single causative agent
Combination of genetic and environmental
influences
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Leukemia
Acute versus chronic
Cell maturity
Acute: Clonal proliferation of immature hematopoietic cells
higher proportion of very immature, nonfunctional cells in
the bone marrow & circulation
Chronic: has higher proportion of mature, WBC and onset
is more gradual, milder signs and thus better prognosis

Acute
Chronic
Chronic leukemia have better prognosis, more
common older person
Classified by cell type
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Leukemias are grouped into 4 types--according to how quickly they progress


and the type of cell involved.
1.) Acute Lymphocytic Leukemia (ALL)**
(malignant cells are mainlyB lymphocytes)
--most common in children
--Signs and symptoms may appear
abruptly
Fever
Bleeding
CNS manifestations, common
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2.) Acute Myeloid(myelogenous) Leukemia (AML)


Myeloblasts affected = precursor to
granulocytes
(malignant cells granulocytes (neutrophils, eosinophils,
basophils)

Also called acute nonlymphoblastic leukemia


(ANLL)
--stem cell of WBC proliferates, decreasing
stem cells availability for RBC and platelets
--result hyperplasia of bone marrow
--25% of all leukemias
85% of the acute leukemias in adults

--Abrupt, dramatic onset


Serious infections or abnormal bleeding
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3.) Chronic Lymphocytic Leukemia (CLL)


--most common form of adult leukemia
( malignant cells are B lymphocytes)
* Lymph node enlargement is present throughout
body
Increased incidence of infection
Pain, paralysis from pressure caused by enlarged lymph
nodes occurs in later stage of disease

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4.) Chronic Myeloid Leukemia (CML)--(malignant cells are granulocytes=


eosinophis, basophils, neutrophils)
*Philadelphia chromosome
Genetic marker
* Move into peripheral blood in massive
numbers
Ultimately infiltrate liver and spleen
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Unclassified Leukemias
Subtype cannot be identified
Malignant leukemic cells may have
characteristics of
Lymphoid
Myeloid
Mixed

Frequently these patients do not respond


well to treatment
Poor prognosis
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Overview: Leukemias

Etiology and Genetic Risk:

Clinical Findings: caused by bone marrow failure and


formation of leukemic infiltrates

1. Bone marrow overcrowding by


abnormal cells
2. Inadequate production of normal
marrow elements
3. Predisposition to anemia,
thrombocytopenia, decreased number
and function of WBCs
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Clinical Manifestations
Laboratory Assessment
To diagnose and classify
Peripheral blood evaluation
Bone marrow evaluation
Definitive test: examination
of cells obtained from
bone marrow aspiration
and biopsy

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Leukemia
Collaborative Care
Goal is to attain remission Infection protection
Complete, partial, or molecular
remission
Bleeding precautions
Chemotherapeutic treatment
Energy management
Four stages of therapy

Induction
Intensification
Consolidation
Maintenance

Drug therapy
Combination chemotherapy
Mainstay treatment
Three purposes
Drug resistance
Drug toxicity using
multiple drugs
Interrupt cell growth
at multiple points

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CHEMOTHERAPY
4 phases:
1st phase =induction
therapy,

--Patient may
become critically ill
Provide
psychological
support as well

2nd phase Intensification


--Intensification therapy

High-dose therapy
May be given after
induction therapy
Same drugs at
higher doses
and/or other drugs

--common S/E=

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3rd phase
therapy=Consolidation
therapy, another course of
chemotherapy single or
repeated for 1-2 years
--intent is to cure
--occurs after remission is
achieved
Eliminate remaining
leukemic cells that
may not be
pathologically evident

4th phase therapy=


Maintenance purpose
is to maintain remission.
--Lower doses of the
same drug
--Goal is to keep the
body free of leukemic
cells
--Not all leukemia
respond to
maintenance therapy

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Collaborative treatments
for Leukemia
Drug therapy for infection
may include antibacterial,
antiviral, or antifungal.

**serious adverse effects


include
ototoxicity (disruption
of hearing and or
balance), nephrotoxicity
(disruption of kidney
function)

Analgesics
Radiation to areas of
lymphocytic infiltrates
Transfusions of whole blood
or blood fractions
Monitor vital signs and assess
for fever. A temperature
elevation of 0.5 degrees F
above baseline is significant
for a client with leukopenia
and indicates an infection
until proven otherwise.

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Collaborative treatments for Leukemia

Bone Marrow Transplantation (Stem


cell)
new healthy bone marrow is given to the
client, which begins the process of
hemotopoiesis, which results in normal,
properly functioning cells, and ideally, a
permanent cure.
a.) Allogenic
b.) Autologous
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c.) Syngeneic bone marrow from human


leukocyte antigen (HLA)-matched identical
twin
Success depends on the accuracy of the tissue
match using human leukocyte antigen (HLA)
Chemotherapy and radiation are used to
prepare the recipients bone marrow for
transplantation
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Bone Marrow Transplantation


Procedure:

Conditioning regiment
before transplantation
Purpose Donor stem cells infused
intravenously into the
1.)
blood of the recipient;
2.)
Antirejection
Engraftment
medications for one
successful take of
year and then
transplanted cells in
discontinued.
clients bone marrow.
Common complications:
8-12 days if occurs
WBC, RBC, and platelet
count begin to rise
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Bone Marrow Transplantation:


overview
Standard treatment for leukemia
Purges present marrow of the leukemic cells
After conditioning, new, healthy marrow
given to the client toward a cure
Sources of stem cells
Conditioning regimen
Transplantation

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Risk for Injury


Nadir: period of
Infection is a major cause of death
greatest bone marrow sepsis is a common
complication.
suppression
Frequent handwashing
Bleeding precautions Private room
HEPA (high efficiency particulate air)
Fatigue
filtration or laminar airflow system
Mask for visitor with upper
Interventions:
respiratory infection
Diet therapy
Minimal bacteria diet without
Blood replacement
therapy
Drug therapy
Energy conservation

uncooked foods
Monitoring of daily laboratory
results
Assessment of vital signs
Skin care, respiratory care

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Nursing Measures for clients with immunocompromised


client

Place in private room whenever possible


Clean room daily
Good hand washing before and after care
Limit using common equipment if at all possible. Keep frequently use
equipment in the room. Utilize disposable eating utensils.
Limit number of health care workers entering the clients room.
Inspect IV sites, wounds, for signs of infection. Obtain cultures if suspect.
Assist client with TCDB
Limit visitors to health adults, and to a minimum.
Use aseptic technique for all invasive procedures
Monitor WBC, ANC daily
VS every 4hrs
Avoid use of indwelling catheters, or other invasive lines, procedures
Keep fresh flowers, fruits, vegetables and potted plants out of the clients
room.
assist and inspect with oral hygiene daily
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Nursing Measures for clients with Leukemia or the


client undergoing bone marrow, stem cell
transplant
Infection protection (pg 715 )
Bleeding precautions (pg. 707)
Energy management (pg. )
Home care of central venous catheter
(pg. )

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Malignant Lymphomas
Lymphomas are cancers (malignancies) of one type of
WBC called lymphocytes. Lymphomas are solid
tumors involving lymph nodes in lymphatic system and in
blood-forming organs
The 2 main types of lymphoma are Hodgkin's lymphoma
(HL) and non-Hodgkin's lymphoma (NHL).
Non-Hodgkin's lymphoma is more common than
Hodgkin's lymphoma

diagnosis and staging of disease is based on a


biopsies, CTs abd/chest, LFTs, bone marrow biopsies

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Lymphomas
Lymphatic system- network of lymphatic vessels
are lymph nodes, which house collections of
lymphocytes.
Lymphocytes that become cancerous
(lymphoma cells) may remain confined to a
single lymph node or may spread to the bone
marrow, the spleen, or virtually any other organ
Lymphomas can develop from either B or T
lymphocytes.
T lymphocytes are important in regulating the
immune system and in fighting viral infections.
B lymphocytes produce antibodies.

the lymphatic system


FCN: transports fluids throughout the body.
consists of thin-walled lymphatic vessels,
lymph nodes, and two collecting ducts
The fluids contain proteins, minerals,
nutrients, and other substances, which
provide nourishment to tissues.
important functions of the lymphatic
system are to remove damaged cells from
the body and to provide protection against
the spread of infection and cancer.

1.) Hodgkins Lymphoma (Reed-Sternberg


specific cancer cell type) is one of the most
curable types of cancer.
occurs in people in their mid to late 20s and in
people older than 50
causes unknown, factors in development may
include viral infection (EBV), and exposure to
chemicals, toxins
2.) Non Hodgkins Lymphoma (12 subtypes)
sixth most common cause of cancer in the United
States
more common in men, white individuals, and people
older than 50 years of age.
Causes: see above, immunosuppressive medications,
chemo, radiation, exposure carcinogens
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Hodgkins Lymphoma
Cancer that starts in a single lymph node or a
single chain of nodes, eventually infiltrates
other organslungs, spleen, liver
Marker: Reed-Sternberg cell
Signs and Symptoms: Large, painless lymph
node usually in the neck, axillary, or inguinal,
fever, malaise, night sweats, weight loss, fever
Other s/s depends on stage of disease
One of the most curable cancers
Disease below diaphragm can spread to liver,
above diaphragm confined to lymph nodes for
a time.
Treatment: external radiation alone or with
combination chemotherapy
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Non-Hodgkins Lymphoma
All lymphoid cancers that do not have the ReedSternberg cell
More than 12 types of non-Hodgkins lymphoma
Burkitts, Large B Cell lymphoma highly aggressive
diseases
Low-grade lymphomas less responsive to treatment;
cures are rare
Treatment: radiation therapy and multi agent
chemotherapy, or single-agent therapy

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Staging of Hodgkin Lymphoma


and Non-Hodgkin
I In 1 lymph region only
II In 2 lymph regions on the same side of
the diaphragm
III In the lymph nodes, spleen, or both and on
both sides of the diaphragm
IV Extranodal involvement (eg, bone marrow,
lung, liver)

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Multiple Myeloma (plasma cell myeloma)


Plasma cell disorder, white blood cell cancer
that involves an overgrowth B lymphocyte cell
= damages bone marrow & bone
excess of antibodies and excess cytokines
causes a progressive bone destruction,
bleeding problems, kidney failure,
immunosuppression.
Multiple tumors develop in vertebrae, ribs,
pelvis, & skull
L/T pathologic or spontaneous FX,
hypercacemia develops D/T bone breakdown
Uncommon cancer
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Cause: unknown, radiation and chemical


exposure may be risk factors
Manifestations: fatigue, easy bruising, bone
pain, fractures, hypertension, increased
infection, hypercalcemia, and fluid imbalance
Diagnosis: bone marrow biopsy (thinning,
Swiss cheese) Bence-Jones protein in
urine
Treatment: chemotherapy, bone marrow
transplant, pain management, bone fracture
prevention, blood transfusion, hydration
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Platelet Disorders & Blood


Clotting Disorders

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Autoimmune
Thrombocytopenic Purpura ITP
Idiopathic thrombocytopenic purpura prior name
Autoimmune
disorder, autoantibodies are
produced
Thrombocytes (plateletes) are recognized as
foreign causing macrophages to destroy them
-- trigger unknown
Bone marrow production of platelet is normal,
platelet destruction is increased, leading to
low number of circulating platelets

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Autoimmune
Thrombocytopenic Purpura

Manifestations: Large ecchymosis or


petechial rash on arms, legs, upper chest, and
neck
Severe complications may include renal failure,
MI, stroke
Diagnosed: by decreased platelet count and
large numbers of megakaryocytes in the bone
marrow,
antiplatelet antibodies may be present

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Autoimmune
Thrombocytopenic Purpura

Interventions (Continued)
include:

Therapy to prevent bleeding


Drug therapy to suppress immune function
(corticosteroids, Imuran)
Blood replacement therapy (platelet
transfusion especially with counts less than
20, 000/cubic mm
Maintain safe environment to reduce risk
bleeding
Splenectomy
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Thrombotic Thrombocytopenic
Purpura (TTP)
Rare disorder; platelets clump abnormally together
in the capillaries, results in bleeding problems
elsewhere, pt. may have hemolytic d/o, neurologic
abnormalities,
Inappropriate clotting when trauma occurs
Cause: Autoimmune reaction in blood vessels
causes clumping, certain drug toxicities
Results in tissue ischemia.
Manifestations: renal failure, MI, stroke
Can be fatal if untreated in 3 months
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Thrombotic Thrombocytopenic
Purpura (TTP)
Collaborative management:
Stop autoimmune process to prevent clotting and
clumping, and decreased platelets circulating (bleeding)
Plasma pheresis, fresh frozen plasma transfusion
Drugs: platelet inhibitors ASA, Prostin, Plicamycin
(antineoplastic, antibiotic)

Platelets generally contraindicatedmay lead to new


vWF platelet complexes and increased clotting
Immunosuppressants

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Heparin-induced thrombocytopenia (HIT)


with or without thrombosis (HITT) white clot
syndrome

is thrombocytopenia (low platelet counts) due to the


administration of heparin

mainly associated with unfractionated heparin


(UFH), it can also occur with exposure to
low-molecular weight heparin (LMWH), at lower
rates
Despite the low platelet count, it is a thrombotic
disorder, with very high rates of thrombosis, in
the arteries with or without venous complications
DVT most common complication
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Typically occur after about five days of


therapy
can appear sooner in patients
previously treated with heparin.
Associated disorders: DVT, DIC,
pulmonary embolism, cerebral
thrombosis, myocardial infarction, and
ischemic injury to the legs or arms,

Type I
Patients have a transient decrease in platelet
count without any further symptoms.
Type II
Cause: autoimmune /allergic reaction to heparin
After heparin is administered to a patient, an
immune complex can form between heparin and
a specific blood factor (platelet factor 4, or "PF4")
that is released by platelets.

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The body views this "heparin-PF4"


complex as a foreign substance.
Therefore, an antibody is formed against
the heparin-PF4 complex.
The antibody binds to this complex and
the platelets are destroyed

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Heparin-induced thrombocytopenia (HIT)


with or without thrombosis (HITT)

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This disruption of platelets can lead to the


formation of new blood clots in patients with
immune-mediated HIT.
The result can be a deep vein thrombosis (in
the veins of the thigh or pelvis), pulmonary
embolism, or even a heart attack or stroke.
--Clot formation is mainly arterial and rich in
platelets ("white clot syndrome")
--L/T thrombocytopenia and platelet-fibrin
thrombi
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DVTs especially in lower extremities may


occur
skin lesions and necrosis may also occur
at the site of the heparin infusion
HIT occurs more commonly in surgical
settings rather than non-surgical settings

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Diagnosis
--decreased platelet counts
--antibody for HIT
Treatment
--D/C Heparin, use other drugs
--Refludan (lepirudin), argotroban (thrombin
inhibitors)
--Fondaparinux

(Arixtra), (Factor X inhibitor)

Severe clotting problemsplasmapheresis


Protamine sulfate antidote to Heparin
Splenectomy
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Hemophilia and Von Willebrand disease

Hemophilia It is an X-linked recessive


bleeding disorder. Hemophilia is a bleeding
disorder caused by a deficiency in one of two
blood clotting factors: factor VIII or factor IX.
There are two forms of hemophilia.
Hemophilia A (classic hemophilia), which
accounts for about 80% of all cases, is a
deficiency in clotting factor VIII.

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Hemophilia B (Christmas disease) is a


deficiency in clotting factor IX.
They are recessive sex-linked, which means
that the gene abnormalities are inherited
through the mother and that nearly everyone
with hemophilia is male.

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Von Willebrand Disease


vWF, a genetic blood disorder that affects
clotting
This glycoprotein is deficient or defective
in von Willebrand disease
L/T variable factor VIII deficiencies
and platelet dysfunction
Autosomal dominant, seen in both sexes

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Diagnosis: development of a large cephalohematoma


or intracranial hemorrhage after birth, a history of
prolonged bleeding after trauma could be indicative
of clotting disorder that needs further evaluation.
Treatment: hemophilia is not curable, but treatments
that includes cryoprecipitate, or replacement of factor
VIII, or IX , DDAVP (synthetic vasopressin stimulates increase in
factor VIII, VWF) to increase clotting factor levels can
prevent crippling deformities and prolong life
expectancy.
--Antifibrinolytic therapyCyclokapron, Amicar

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Disseminated Intravascular Coagulation(DIC)

Involves both : Serious bleeding and


thrombotic (clotting) D/O
DIC is a life threatening disorder
that occurs as a complication of
diseases and conditions that
accelerate clotting, L/T depletion of
platelets & clotting factors and
eventually L/T uncontrollable
hemorrhage occurs.
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Disseminated Intravascular Coagulation(DIC)

A. Causes:
infection, septecemia, viral, fungal,
rickettsial, protozoal infections, obstetrical
complications, neoplastic diseases, burns,
trauma, heatstroke, or any other number of
disorders.

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B. Pathophysiology: unclear why certain


disorders lead to DIC. The triggering
mechanism is by the entrance of a foreign
protein (infection) , particularly gram-negative
infection
L/T endotoxins into the circulation and cause
vascular endothelial vessel injury
(microvascularcirculation) L/T trigger clotting
mechanism throughout body-L/T comsumption
of clotting factors and fibrinolysis occurs
L/T hemorrhage, and eventually hypotension &
shock

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Accelerated clotting, activation of


prothrombin, excess thrombin
Thrombin converts fibrinogen to fibrin
Fibrin clots in microcirculation
Hypofibrinogenia, hypoprothrombinemia,
thrombocytopenia, deficiencies in factors
V and VIII
Fibrinolytic system activates dissolves
fibrin clots
HEMORRHAGE is result of fibrin
degradation products, depletion of plasma
coagulation factors
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DIC
Diagnostic Tests
PTT/PT prolonged
Reduced fibrinogen, antithrombin, and
platelets
Elevated fibrin-split products, and elevated
D-dimers (cross linked fibrin fragments)
Decreased levels of factors V, VII, VII, X,
XIII

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Disseminated Intravascular
Coagulation(DIC)
Clinical Findings:
abnormal bleeding anywhere in the body,
cutaneous oozing of serum, cyanotic
extremities, petechia, restlessness, anxiety
Complications: acute tubular necrosis, shock,
multi organ failure
Interventions/Treatment: treatment of
underlying disorder, administration of blood,
fresh frozen plasma, platelet, packed RBCs,
heparin therapy (controversial)
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