HEMATOLOGIC DISORDERS
HEMATOLOGY
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The hematologic or hematopoietic system includes
the blood, blood vessels, and blood forming organs (
bone marrow, spleen, liver, lymph nodes, and thymus
gland).
•
Major function of blood is to carry necessary materials
( oxygen, nutrients ) to cells and to remove CO2
system and metabolic waste products.
•
It also plays a role in hormone transport, inflammatory
and immune responses, temperature regulation, fluidelectrolyte balance, and acid-base balance.
THREE BROAD FUNCTIONS OF BLOOD :
Transportation
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Respiratory – transport of gases by the RBC
•
Nutritive – transport of digested nutrients
from the GIT to the different cells of the body
•
Excretory – transport of metabolic wastes to
the kidneys and excreted as urine
Regulation
•
Hormones and other molecules that help
regulate metabolism are also carried in the
blood
•
Thermoregulation
Protection
•
Blood clotting
•
Leukocytes
COMPONENTS OF THE BLOOD :
1. Plasma
2. Formed Elements
•
Erythrocytes or RBC
•
Leukocytes or WBC
•
Thrombocytes or Platelets
BLOOD
•
Average volume is 5 – 6 liters or approximately 6
quarts
•
pH is 7.35 – 7.45
•
Arterial blood is usually bright red in color compared
to venous blood which has a darker color, due
primarily to the large concentration of oxyhemoglobin
found in arterial blood
BLOOD
a. ) Plasma - fluid portion of the blood
Contains :
proteins / albumin
fibrinogen
clotting factors
electrolytes
waste products
nutrients
b. ) Cellular Components
1. Leukocytes (WBC)
2. Erythrocyte (RBC)
3. Thrombocyte (Platelets)
Hematopoiesis – occurs in the bone marrow ( pelvis, ribs,
vertebrae and sternum.
Extramedullary Hematopoiesis - the liver and the spleen
produces blood cells
PLASMA
•
•
•
The liquid part of the blood
Approximately 90% water
Also contains nutrients, ions (salts, primarily Na),
respiratory gases, hormones, plasma proteins,
•
1.
2.
3.
antibodies and various wastes and products of
cellular metabolism
PLASMA PROTEINS – the most abundant solutes in
the plasma
Three Types
Albumin
Globulin
Fibrinogen
FORMED ELEMENTS OF THE BLOOD
Erythrocytes
•
Also called Red Blood Cells or RBC’s
•
Function primarily to ferry Oxygen in the blood to all
cells in the body
•
Also transports Carbon dioxide out of the body
•
Lifespan of 120 days only
•
Hemoglobin in the RBC binds with the Oxygen as it is
transported in the blood
•
Female : 12 – 16 g/100ml
•
Male
: 13 – 18 g/100ml
•
Normal RBC count: about 4 – 6 million/mm³
•
Hematocrit (HCT) – percentage of RBC per given
volume of blood and is an important indicator of the
Oxygen-carrying capacity of the blood
•
Female : 37 – 48%
•
Male
: 45 – 52%
Leukocytes
•
Also called White Blood Cells or WBC’s
•
Average value : 4,000 – 11,000 / mm³
•
Protects the body against any damage
•
Are able to slip in and out of the blood vessels by
ameboid fashion – in a process called diapedesis
•
When mobilized, the body speeds up production
which usually indicates the presence of infection in
the body
•
Leukocytosis – total WBC count above 11,000 / mm³
•
Leukopenia – an abnormally low WBC count
•
Types:
o Granulocytes

Neutrophils
Eosinophils
Basophils
o Agranulocytes

Lymphocytes
Monocytes
Cells of the Immune System
•
Lymphocytes
•
Lymphocytes are created in the bone
marrow and migrate to the Thymus where
they mature
•
After becoming immunocompetent, the B & T
cells transfer to the lymph nodes & spleen
•
Types
1. B lymphocytes or B cells –
produces antibodies to incapacitate
the antigen
2. T lymphocytes or T Cells – attacks
antigens directly
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Macrophages
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Literally means “Big Eaters”
•
Arise from monocytes formed in the bone
marrow
•
Major role : to engulf foreign particles
•
Cellular (Cell-Mediated) Immune Response
•
T – Cells
•
Responds directly to antigens
•
Will destroy target cells thru secretions of
Lymphokines and Perforin ( “Kiss of Death”)
which is inserted to the cell membrane,
shortly after that, the target cell ruptures
•
They have a:
•
License to KILL
•
License to HELP
•
License to Suppress
•
Three types :
•
Killer T Cells – binds to the surface
of invading cells, disrupt the cell
membrane & destroy it by altering
it’s environment
•
Helper T cells – helps to stimulate
the B Cells to mature into Plasma
Cells which synthetize & secrete
immunoglobulins (Antibodies)
•
Suppressor T Cells – Reduces the
Humoral response
•
Humoral (Antibody-Mediated) Immune Response
B – Cells
•
Matures into Plasma Cells responsible for
Antibody production
Cell Type
Normal Value / mm³
5 Classes of Immunoglobulins (MADGE) :
1. Immunoglobulin M (IgM)
1st immunoglobulin produced in an immune response
present in plasma, too big to cross membrane barriers
2. Immunoglobulin A (IgA)
Sound in body secretions like saliva, tears, mucus,
bile, milk & colostrum
3. Immunoglobulin D (IgD)
Present only in the plasma & is always attached to the
B Cell
4. Immunoglobulin G (IgG)
80% of circulating antibodies
Can cross the placenta and provide passive immunity
Present in all body fluids
5. Immunoglobulin E (IgE)
Responsible for Allergic & hypersensitivity reactions
Stimulates Mast cells & Basophils to release
Histamine which mediates inflammation & the allergic
response
Thrombocytes
•
Also called Platelets
•
Average value : 250,000 – 450,000 / mm³
•
Lives for about 5 – 10 days
•
Important in blood clotting
F u n c ti o n
RBC
4 – 6 million
WBC
4,000 – 11,000
Neutrophils
3,000 – 7,000
40 – 70% of WBC
Active Phagocytes, increases during acute
infections
Eosinophils
100 – 400
1 – 4% of WBC
Kills parasitic worms, increases in allergy
attacks, helps detoxify foreign substances
Basophils
20 – 50
0 – 1% of WBC
Contains histamine
Releases anticoagulant heparin
Lymphocytes
1,500 – 3,000
20 – 45% of WBC
Part of Immune System
B & T Lymphocytes
Monocytes
100 – 700
4 – 8% of WBC
Phagocytes that become macrophages –
“clean-up team” & increases in chronic
infections
Platelets
250,000 – 450,000
Needed for Blood Clotting
Initiates Clotting Cascade
Hematopoiesis (Blood Cell Formation)
•
•
•
•
•
•
Occurs in the Red Bone Marrow, chiefly in flat bones
like Skull, ribs, pelvis, sternum and proximal
epiphyses of the humerus and femur
Erythropoiesis – RBC production, is a very active
process
RBC are continuously being destroyed by the liver &
spleen
RBC’s have a lifespan of 120 days
As RBC’s are destroyed, iron is recycled to the bone
marrow for use in the formation of new RBC’s
Erythropoietin – secreted by the kidneys & released
when blood levels of Oxygen begins to decline for any
Transport Oxygen
reason; which stimulates the Red Bone Marrow to
produce more RBC’s
ERYTHROCYTES
> destruction
- mature cells removed chiefly by spleen &
liver
* BILIRUBIN = byproduct of Hgb released when
RBC’s destroyed
* IRON = freed from Hgb during bilirubin formation
= transported to bone marrow via
TRANSFERIN & reclaimed for new Hgb production
Glucuronic
acid
ERYTHROPOIESIS - RBC production
Requirements :
•
a. Erythropoietin
b. Iron
c. Folic Acid
d. Vitamin B6, Vitamin B12
e. Vitamin C
Liver and Spleen - Graveyard of the RBC
•
•
plasma Clotting Factors form an
activator that triggers the Clotting
Cascade
a Blood Clot is formed
Serum is squeezed out within the
hour pulling the ruptured edges
together
Hemostasis (Blood Clotting)
•
Three Major Phases
1. Platelet Plug Formation
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Platelets adhere and stick to vessel
lining that are damaged forming a
Platelet Plug or White Thrombus
•
Platelets release chemicals to
attract more platelets to the injured
site
2. Vascular Spasms
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Platelets release Serotonin causing
spasms of the blood vessel,
constricting it & decreasing blood
flow
3. Coagulation or Blood Clotting
•
Thromboplastin is released by
damaged cells
Plasma Clotting Factors
I
II
III
IV
V
VII
VIII
IX
X
XI
XII
XIII
Fibrinogen
Prothrombin
Tissue Thromboplastin
Calcium
Proacelerin
Proconvertin
Antihemophilic Factor
Christmas Factor
Stuart – Prower Factor
Plasma Thromboplastin Antecedent
Hageman Factor
Fibrin Stabilizing Factor
Anemia
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Conditions in which the number of RBC’s or amount of hemoglobin is lower than normal
•
leads to hypoxia and ischemia
•
Classifications of Anemia According to Etiology
Bleeding
Hypoproliferative
Hemolytic
Resulting from RBC Loss
low RBC production
increased RBC destruction
accidents / trauma
Iron deficiency
enlarged spleen
Surgery
Vit. B12 deficiency
sickle cell
Childbirth
Folic acid deficiency
thalassemia
ruptured blood vessel
Vit. C deficiency
G6PD
menorrhagia
chronic disease
drug-induced
Epistaxis
low erythropoietin
hemorrhoids
cancer
GI bleeding / ulcers
Cancer
Iron Deficiency Anemia
•
most common type of anemia
•
Iron stores are depleted, resulting in a decreased supply of iron for the manufacture of hemoglobin in RBC’s
Commonly results from blood loss, increased metabolic demands, syndromes of gastrointestinal malabsorption, and dietary
inadequacy
•
cause : inadequate absorption or excessive
loss of iron
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Bleeding – principal cause in adults
•
Vegetarian diets
•
Vitamin C – increases iron absorption
Pathophysiology
Stage 2
Stage 1
Iron loss exceeds intake,
depleting iron reserves
Fewer RBC are produced
Stage 3
Anemia develops
RBC – normal but few in number
Hgb & Hct – normal levels
Stage 4
Bone marrow compensates :
Speeds up production – Microlytic /
Small red blood cells
Stage 5
Symptoms of anemia worsens
Most definitive way to diagnose anemia : Bone marrow
aspiration
Assessment Findings
Mild cases - asymptomatic
fatigue
dyspnea
Palpitations & dizziness
Pallor
Brittle hair and nails
Pica
Glossitis
Cheilosis
Irritability
Koilonychia
Laboratory findings :
RBC’s are small / microcytic and pale
•
decreased hemoglobin
decreased
hematocrit
•
decreased serum iron
decreased ferritin
Nursing Interventions
1. Identify the cause
2. Monitor S/Sx of bleeding – stool, urine and GI contents
3. Provide rest
4. Give iron preparations ( 6 – 12 months )
Ferrous Sulfate
Ferrous
Gluconate
Ferrous
Fumarate
a. always give after meals or snacks
b. dilute liquid preps and give thru straw
c. give with orange juice (Vitamin C enhances absorption)
d. warn clients the stool will become black and can cause
constipation
5. For clients with poor absorption or continuous blood loss
IM or IV of Iron Dextran
a. Use 1 needle to withdraw and another for injection
b. Use z-track method
c. don’t massage but encourage ambulation
d. usually, deep IM at buttocks
6. Give dietary teaching – liver, meats, nuts, egg yolk, shellfish,
legumes, etc.
7. Increase intake of roughage and fluids to prevent
constipation.
Pernicious Anemia
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Vitamin B12 Deficiency Anemia
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caused by inadequate Vit. B12 intake or
deficiency in intrinsic factor
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Vit. B12 combines with intrinsic factor so it can be
absorbed in the ileum into the bloodstream`
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the result is abnormally large erythrocytes and
hypochlorhydria ( a deficiency of hydrochloric acid in
gastric secretions).
•
Lack of intrinsic factor is caused by gastric
mucosal atrophy (possibly due to heredity, prolonged
iron deficiency, or an autoimmune disorder), can also
result in client who have had a total gastrectomy
•
Usually occurs in men and women over age 50,
with an increase in blue eyed persons.
PATHOPHYSIOLOGY
Intrinsic factor + Vit. B12 for absorption
Result : decreased or no Vit. B12
Lead to : decreased RBC production
Assessment :
Anemia - symptoms are :
Fatigue, weakness
dyspnea
Palpitations & dizziness
Pallor
confusion
Decreased intellectual fxn
Sore tongue : Beefy red tongue
Paresthesias
Weight loss
Lab Results
Decrease RBC
Decreased free Hydrochloric acid
Large RBC / Megaloblast
Positive Schilling Test – definitive test for Pernicious anemia
- used to detect lack of intrinsic
factor
Positive schilling test
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Measures absorption of radioactive vitamin B12
both before and after parenteral administration of
intrinsic factor.
•
Definitive test for pernicious anemia.
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Used to detect lack of intrinsic factor.
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Fasting client is given radioactive vitamin B12 by
mouth and nonradioactive vitamin B12 IM to saturate
tissue binding sites and to permit some excretion of
radioactive vitamin B12 in the urine if it is absorbed.
•
24-48 hour urine collection is obtained; client is
encouraged to drink fluids.
•
If indicated, a second stage Schilling test will be
performed 1 week after first stage.
•
Fasting client is given radioactive vitamin B12
combined with human intrinsic factor and the test will
be repeated.
Nursing Interventions / Treatment
1. Drug Therapy
a. Vit. B12 injections monthly for life
b. Iron Preparations
c. Folic Acid
2. Transfusion therapy
3. Bed rest
4. Mouth care
5. Dietary teaching
6. Teach about importance of lifelong Vitamin B12 therapy
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Provide a nutritious diet high in iron, protein, and
vitamins (fish, meat, milk/milk products and eggs).
•
Avoid highly seasoned, coarse or very hot foods if
client has a mouth sores.
•
Provide mouth care before and after meals using a
soft toothbrush and non irritating rinses.
HEMOLYTIC ANEMIAS
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increase rate of RBC destruction
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short life span of RBC.
TYPES:
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G6PD
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Sickle cell anemia
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Thalassemia
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DIC
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Transfussion incompatibilities
Sickle Cell Anemia
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Most common inherited disease among black
Americans.
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Also found in Arabian, Mediterranean and Caribbean
descent
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Hgb S ( abnormal hemoglobin ), which has reduced
oxygen carrying capacity, replaces all or part of the
hemoglobin in the RBC’s.
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Life span is 6-20 days instead of 120, causing
hemolytic anemia.
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Death often occurs in early adulthood due to
occlusion or infection.
•
During decreased O2 tension, lowered pH,
dehydration and severe infections, RBC’s change
from round to sickle or crescent shape
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Sickled cells don’t slide thru vessels as normal RBC’s
do, causing clumping, thrombosis, arterial obstruction,
increased blood viscosity, hemolysis and eventual
tissue ischemia and necrosis
Sickle Cell Crisis :
•
Cause : infection, dehydration, fever, cold exposure,
hypoxia, strenuous exercise, extreme fatigue or
extreme changes in altitude
VASO-OCCLUSIVE CRISIS:
A. most common and most painful type of crisis
•
caused by stasis of blood with clumping of the cells in
the microcirculation leading to ischemia & infarction.
B. signs include fever, pain, and tissue engorgement
C. Treatment
– hydration, electrolyte replacement, bed
rest, broad spectrum antibiotics, transfusions and oxygen
therapy.
SPLENIC SEQUESTRATION:
A. Life - threatening crisis caused by the pooling of blood in the
spleen. (from congestion of sickled cells)
•
•
B. signs include profound anemia, hypovolemia, and shock
C. treatment: blood transfusions and splenectomy
APLASTIC CRISIS:
A. Occurs infrequently and is caused by:
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diminished production of RBC
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increased destruction of RBC’s
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triggered by a viral infection or the
depletion of folic acid.
B. signs include profound anemia, pallor, and
PANCYTOPENIA.
C. Treatment – Transfusion of packed RBC’s
•
•
Frequent infection esp. with H. influenzae
Infants may have Dactylitis (hand – foot syndrome)
symmetrical painful soft tissue swelling in the hands
and feet in the absence of trauma
ASSESSMENT
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Signs and symptoms of anemia – pallor, weakness
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Hepatosplenomegaly
•
Dactylitis (Symmetric swelling of the hands and feet)
– called hand-foot syndrome
•
Other problems :
– CVA
– MI
– Growth retardation – initial manifestation
– Decreased fertility
– Priapism
– Recurrent severe infections
MEDICAL MANAGEMENT
A. Drug therapy
> analgesic/narcotics to control pain
•
Avoid meperidine (Demerol) due increased risk of
seizures in children
> antibiotics to control infection.
B. Blood transfusions
C. Hydration:oral and IV
D. Bed rest
E. Surgery: splenectomy
INTERVENTIONS
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Administer O2 & Blood Transfusion as Rx
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Maintain adequate hydration
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Avoid tight clothing that could impair circulation.
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Keep wounds clean and dry.
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Provide bed rest to decrease energy expenditure and
oxygen use.
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Encourage patient to eat foods high in calories,
CHON, with folic acid supplementation.
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Analgesics:
•
Acetaminophen
•
Endocrine abnormalities :
– delayed development of secondary sex
characteristics – most boys fail to undergo
puberty, girls – menstruation problems
– DM – due to iron deposits in the pancreas
– Hypermetabolic rates
•
Skeletal complications
– Frontal & parietal bossing (Enlargement)
–
Maxillary hypertrophy – leading to occlusion
– Premature closure of epiphyses of long
bones
– Osteoporosis & pathologic fractures
•
Cardiac problems: pericarditis & CHF – usual cause
of death
•
Gallbladder disease
– Gallstones that often require surgery
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Skin – bronze pigmentation caused by iron deposits in
the dermis
•
Leg ulcers
ERYTHROBLASTOSIS FETALIS
Rh Incompatibility
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Destruction of RBCs that result from Ag-Ab rxn
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Characterized by hemolytic anemia or
hyperbilirubinemia
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Possibly caused by Rh incompatibility between the
mother & the fetus (Ag & Ab
reaction)
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Sensitization of Rh (-) woman by transfusion of Rh
(+) blood
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Sensitization of Rh (-) woman by presence of Rh (+)
RBCs from her fetus conceived with Rh (+) man
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•
•
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Morphine
avoid aspirin as it enhances acidosis,which
promotes sickling
Avoid anticoagulants( sludging is not due to clotting ).
Antibiotics.
Avoid activities that require so much energy.
Keep arms and legs from extreme cold.
Decrease emotional stress.
Provide good skin care
THALASSEMIA MAJOR
(Cooley’s anemia)
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B - thalassemia refers to an inherited hemolytic
anemia, characterized by reduction or absence of the
B-globulin chain in Hgb synthesis
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Fragile RBC & short life span
•
Autosomal recessive pattern of inheritance
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Insufficient B-globulin chain synthesis allows large
amounts of unstable chains to accumulate
•
Precipitates of alpha chains that form cause RBC’s to
be rigid & easily destroyed, leading to severe
hemolytic anemia = chronic hypoxia
•
Skeletal deformities: pathologic fractures
•
Hemosiderosis – excess iron supply, which leads to
iron deposits in the organ tissues leading to
decreased function
CLINICAL MANIFESTATIONS
•
onset is usually insidious
•
Sx are primarily related to progressive anemia,
expansion of marrow cavities of the bone &
development of hemosiderosis
•
Early Sx often include progressive pallor, poor feeding
& lethargy
•
Further signs: hemorrhage, bone pain, exercise
intolerance, jaundice, & protuberant abdomen
DIAGNOSTIC EVALUATION
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Decrease hemoglobin
•
RBC= increase in number
•
Hgb elctrophoresis
–
elevated levels of HgF ( doesn’t hold O2 well
)
–
limited amount of HgA
Management
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Frequent and regular transfusion of packed RBC’s to
maintain Hgb levels above 10 g/dL
•
Iron chelation therapy with deferoxamine (Desferal) –
reduces toxic effects of excess iron & increases iron
excretion thru urine & feces
•
Splenectomy
•
Supportive management of symptoms
•
Bone marrow transplant
•
Prognosis and Survival rate is poor because of no
known cure
•
Often fatal in late adolescence or early adulthood
Complications
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Splenomegaly
Growth retardation in the second decade
•
Approximately 65% of infants conceived by this
combination of parents will be Rh (+)
•
Mother is sensitized by passage of Rh (+) RBCs thru
placenta, either during pregnancy (break/leak in
membrane) or at the time of separation of
the placenta after delivery.
RH INCOMPATIBILTY
•
FIRST PREGNANCY
- mother may become sensitized, baby rarely affected
INDIRECT COOMB’S TEST
- Tests for anti-Rh(+) Ab in mother’s circulation
- performed during pregnancy at first visit & again about 28
week’s gestation.
RESULTS:
- If (-) at 28 weeks, a small dose of (MicroRhogam) is given
prophylactically to prevent sensitization in the 3rd trimester.
- Rhogam may also be given after 2nd trimester
amniocentesis
- If (+), levels are titrated to determine potential effects on
the fetus
DIRECT COOMBS’ TEST
- Tests done on the cord blood at delivery to determine
presence of (+) Ab on fetal RBCs
RESULTS
- If both indirect & direct Coombs’ test is NEGATIVE & infant
is Rh(+):
- NEGATIVE: No formation of Anti-Rh (+) Ab
- Rhogam (Rho[D] human immune globulin is given to the
Rh(-) mother to prevent development of anti-Rh(+) Ab as the
result of sensitization from present or just terminated
pregnancy.
•
In each pregnancy, an Rh(-) mother who carries an
Rh (+) fetus receives Rhogam if both the mother and
infant is (-)
to both direct & indirect Coombs’
test.
•
If mother is has been sensitized:
anti-Rh(+) Ab are present
- Rhogam is not indicated
Rhogam must be injected into unsensitized mother’s
system within 72 hours of delivery of Rh(+) infant
CLINICAL FINDINGS
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Anemia
•
Jaundice that develops rapidly after birth and before
24 hours or that occurs within 24 - 36 hours
•
Enlarged placenta
•
Edema
•
Ascites
•
NURSING INTERVENTIONS
•
Determine blood type and Rh early in pregnancy.
•
Determine results of direct Coomb’s test early in
pregnancy & again at 28 week’s.
•
Determine results of direct Coomb’s test on cord
blood.
- type & Rh, Hgb, Hct
•
Implement phototherapy or exchange transfusion.
•
Administer Rh0 (D) immune globulin to the mother
during the first 72 hrs. after delivery if the Rh(-)
mother delivers an Rh (+) fetus but remains
unsensitized
•
Assist with exchange transfusion as prescribed.
•
The baby undergoes transfusion of blood to stop the
destruction of the baby’s RBC
- the transfused blood is replaced with the baby’s own blood
gradually
•
Reassure the mother that the newborn will suffer no
untoward effects
from the condition
MYELOPROLIFERATIVE DISORDER
POLYCYTHEMIA VERA
Chronic myeloproliferative d.o. involves bone marrow elements
increase RBC mass & hgb
•
Underlying cause is unknown
•
Hyperplasia of all bone marrow elements
> increase RBC mass
> increase blood volume viscosity
> decrease marrow iron reserve
> Splenomegaly
ASSESSMENT
•
Reddish purple hue of skin & mucosa, pruritus
•
Splenomegaly, hepatomegaly
•
Epigastric discomfort, abdominal discomfort
•
Painful fingers & toes from paresthesias
•
Altered mentation
•
Weakness, fatigue, night sweats, bleeding tendency
•
Hyperuricemia – from increased RBD formation and
destruction
DX TESTS
•
CBC
•
BONE MARROW ASPIRATION & Biopsy
MANAGEMENT
•
HYPERVISCOSITY
= phlebotomy @ intervals determined by CBC results
to decrease RBC mass
=generally 250-500ml removal @ a time
•
HYPERPLASIA
= myelosuppressive therapy,
= generally using hydroxyurea or IV radioactive
phosphorus (32P), biologic response modifier, ie alpha
interferon
•
HYPERURICEMIA= allupurinol (Zyloprim)
•
PRURITUS = antihistamines (cimitidine), low dose
acetyl salicylic acid; certain anti-depressants
(paroxetin), phototherapy, cholestyramine
INTERVENTION
•
Encourage/assist ambulation
•
Assess for early S/Sx of thromboembolic
complications: swelling of limbs, increased warmth,
pain
•
Monitor CBC & assist with phlebotomy as ordered
Patient Education
•
Educate about risk of thrombosis; encourage patient
to maintain normal activity pattern & avoid long
periods of rest
•
Avoid hot showers
•
Report @ regular intervals for follow up blood
DISORDERS OF PLATELETS AND CLOTTING MECHANISM
HEMOPHILIA
•
Hereditary coagulation defect, usually transmitted to
affected male by female carrier through sex – linked
recessive gene, resulting in prolonged clotting time.
•
Most common type is Hemophilia A or Classic
Hemophilia - factor VIII deficiency (called
Antihemophilic Factor / AHF)
•
Hemophilia B or Christmas Disease – factor IX
deficiency (called the Christmas Factor)
•
Male inherits hemophilia from their mothers, and
females inherit the carrier status from their fathers.
–
Found predominantly, but not exclusive, in
male offsprings
•
Bleeding occurs due to impaired ability to form fibrin
clot
ASSESSMENT
•
Abnormal bleeding in response to trauma or surgery. (muscles/joints)
•
Joint bleeding causing pain, tenderness, swelling, and limited range of motion.
•
Tendency to bruise easily.
•
Epistaxis
•
Hemarthrosis (bleeding in joints causing pain, swelling and limited movement)
IMPLEMENTATION
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Administer factor VIII concentrate.
•
Monitor for bleeding and maintain bleeding precautions.
•
Monitor for joint pain; IMMOBILIZE the affected extremity if joint pains occur.
•
Monitor urine for hematuria.
•
Instruct the parents regarding activities for the child, emphasizing the avoidance of contact sports.
•
Instruct the parents on how to control bleeding (direct/indirect pressure)
•
DDVAP (Desmopressin) – promotes the release of Factor VIII in hemophilia A
•
Use soft toothbrush and point out need for regular dental checkups
•
Refer to National Hemophilia Association
•
Emphasize avoidance of Aspirin
•
Provide diet information as excess weight places further stress on joints
R - Rest
I - Immobilize
C - Cold Compress
E - Elevate
DISSIMINATED INTRAVASCULAR COAGULATION
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DIC is a disorder of diffuse activation of the clotting cascade that results in depletion of clotting factors in the blood.
•
occurs when the blood clotting mechanisms are activated all over the body instead of being localized to an area of injury.
•
grave coagulopathy resulting from overstimulation of clotting & anticlotting processess in response to disease & injury
generalized intravascular clotting which in turn overstimulates fibrinolytic mechanisms
hypercoagulability
hypocoagulability
hemorrhage
•
•
Small blood clots form throughout the body, and eventually the blood clotting factors are used up and not available to form
clots at sites of tissue injury.
Clot - dissolving mechanisms are also increased stimulated by many factors including infection in the blood & severe
tissue injury – burns and head injury, reactions to blood transfusions, carcinomas and obstetrical complications such as
retained placenta after delivery.
ASSESSMENT
•
purpura on lower extremities & abdomen
•
hemorrhagic bullae, acral cyanosis, focal gangrene in skin
Dx Tests:
•
•
•
marked decrease of blood platelets
low levels of fibrinogen & other clotting factors
prolonged prothrombin & partial thromboplastin times & abnormal erythrocyte morphologic characteristics
Nursing Interventions / Treatment
1. To determine the underlying cause of DIC and provide
treatment for it.
2. Replacement therapy of the coagulation factors is achieved
by transfusion of fresh frozen plasma. Cryoprecipitates may
also be used if fibrinogen is significantly decreased. Platelet
transfusions if platelets are diminished
3. Heparin, a medication used to prevent thrombosis, is
sometimes used in combination with replacement therapy. (
still controversial )
4. Prevent further injury
a. avoid IM injections
b. apply pressure to bleeding sites
c. turn patient frequently and gently
d. provide mouth care – soft bristled
toothbrush
5. Teach patient the importance of avoiding aspirin.
IDIOPATHIC THROMBOCYTOPENIA PURPURA
•
Increased destruction of platelets with resultant
platelet count of less that 100,000/mm3 characterized
by petechiae and ecchymoses of the skin.
•
Exact cause unknown; may be autoimmune.
•
Spleen is the site for destruction of platelets
•
often triggered by URTI or Childhood communicable
disease – Measles & chickenpox
ASSESSMENT:
•
Petechiae
•
Ecchymosis
•
Blood in any body secretions, bleeding form mucous
membranes, nosebleeds.
•
Decreased platelet count
•
Anemia
•
easy bruising
•
blood in stool or urine
•
CBC reveals platelet count below 20,000/mm3
•
Bone marrow aspiration done to rule out leukemia
MEDICAL MANAGEMENT:
•
Drug therapy:
•
Prednisone – decreases anti-platelet
antibodies (monitor for infection)
•
IVIG (Intravenous Immune Globulin) – helps
to effectively increase platelet count
•
Anti-D Antibody – one dose treatment
•
Given to pt’s 1 year but less than
19 years old
•
•
•
•
Normal WBC and hemoglobin
no active bleeding present
no concurrent infection
Diphenhydramine and
hydrocortisine are made ready for
possible allergic reactions to the
medication
•
Platelet transfusion
•
Splenectomy
INTERVENTION
•
Prevent, control and minimize bleeding.
•
Prevent bruising
•
Provide support to client and be sensitive to change in
body image.
•
Protect from infection.
•
Administer analgesics (acetaminophen) as ordered;
avoid aspirin.
•
administer meds orally, rectally, or I.V. rather than
I.M.
BLOOD AND BLOOD PRODUCTS
Whole Blood
•
Contents
–
RBC’s
–
WBC’s
–
Platelets
–
Plasma
–
Clotting factors
•
Indications
–
Acute loss of whole blood
Packed Cells
•
Contents
–
RBC’s
–
20% Plasma
•
Indications
–
Replace O2 carrying capacity with less
volume
–
Severe anemia, slow blood loss, CHF
Granulocytes
•
Contents
–
WBC’s
–
20% Plasma
•
Indications
– Life-threatening decreases in WBC count
Plasma
•
Contents
– Clotting factors
– Fibrinogen
– Prothrombin
– Albumin
– Globulins
•
Indications
– Clotting factor deficiency
– Volume expansion
Plasma Protein Fraction
•
Contents
– 5% Albumin/Globin in saline
•
Indications
– Expand volume in burns
– Hemorrhage
– Hypoproteinemia
Albumin
•
Contents
– 5% or 25% albumin
•
Indications
– Replace volume in shock
– Burns
– Hypoproteinemia
Cryoprecipitate
•
Contents
– Factors VIII and XIII, Fibrinogen
•
Indications
– Hemophilia A
– Fibrinogen deficiency
– Factor XIII deficiency
Prothrombin
•
Contents
– Factors II, VII, IX, and X
•
Indications
– Hemophilia B
– Liver disease
Blood Transfusion
•
ABO Antigens
– A Antigen
– B Antigens
– A and B Antigens
– No Antigens
TRANSFUSION COMPLICATIONS
Allergic Reactions
•
Signs/Symptoms
– Itching
– Uticaria
– Chills
– Fever
– Facial edema
– Wheezing
– Anaphylactic shock
•
Management
– Oxygen
– IV fluids
– Epinephrine
– Antihistamines
Hemolytic Reaction
•
Signs/Symptoms
– Chills, fever
– Low back pain
– Headache
– Chest pain
– Dyspnea
– Cyanosis
– Restlessness, anxiety
– Hypotension
– Red urine
•
Management
– Stop transfusion
– Treat shock
– Volume replacement
– Mannitol
Volume Overload
•
Signs/Symptoms
– Cough
– Chest pain
– Dyspnea
– Distended neck veins
Type A
Type B
Type AB
Type O
–
Rales
–
Frothy sputum
•
Management
–
Slow infusion
–
Diuretics
–
Vasodilators
Transfusion Complications
•
Coagulation Disturbances
–
Platelet/Clotting factor deterioration
•
Citrate Intoxication
–
Hypocalcemia
–
Metabolic Alkalosis
•
Hyperkalemia
–
RBC’s Lyse/Release K+
•
Hypothermia
–
Inadequate warming during transfusion
•
Viral Hepatitis
–
Risk rises with each unit
Blood Transfusion
•
IV catheter 18g or larger
•
No fluid other than saline
–
D5W lyses RBC’s
–
LR contains calcium/triggers clotting
•
Two persons confirm ABO/Rh
•
Blood filter in administration set
•
Infusion pumps
–
Excessive pressure can cause hemolysis
•
Rewarming above 380C can cause hemolysis
•
Never add medications directly
Plasma Clotting Factors
I
II
III
IV
V
VII
VIII
IX
X
XI
XII
XIII
Fibrinogen
Prothrombin
Tissue Thromboplastin
Calcium
Proacelerin
Proconvertin
Antihemophilic Factor
Christmas Factor
Stuart – Prower Factor
Plasma Thromboplastin Antecedent
Hageman Factor
Fibrin Stabilizing Factor