Transcript Hematological System Alterations Updated Fall 2011

Hematological System
Alterations
Diane L. McLean, RN, BSN, MSN
Nursing 202
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Learning Objectives:
• Define terms associated with selected
hematological alterations.
• Describe selected hematological
alterations.
• Describe the pathophysiology for
elected hematological alterations.
• Describe the role of the nurse in
providing care for patients experiencing
selected hematological alterations.
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Learning Objectives…
• Explain health promotion related to
selected hematological alterations.
• Interpret clinical manifestations of
selected hematological alterations.
• Interpret diagnostic tests for selected
hematological alterations.
• Describe the pharmacological agents
and/or treatments for selected
hematological alterations.
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Learning Objectives…
• Evaluate nutritional considerations for
patients experiencing selected
hematological alterations.
• Identify expected outcomes of treatment
modalities for patients experiencing
selected hematological alterations.
• Evaluate psychosocial needs of
patients, families, and/or support
systems.
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Learning Objectives…
• Use critical thinking to manage nursing
care for culturally diverse patients
experiencing selected hematological
alterations.
• Evaluate expected outcomes of nursing
care for patients experiencing select
hematological alterations.
• Assess the impact of selected
hematological alterations on maternal
and pediatric patients.
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Anatomy and Physiology of Liver
• The largest organ in the body
• Produces bile, which emulsifies fat and stimulates peristalsis
• Conveys bile to the duodenum at the sphincter of Oddi through the
common bile duct
• Metabolizes carbohydrates, fats and proteins
• Synthesizes coagulation factors VII, IX, X and prothrombin
• Stores Vitamin A, D, B12, and Iron
• Detoxifies chemicals
• Excretes bilirubin
• Receives dual blood supply from portal vein and hepatic artery
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Accessory Organs of Blood
Formation
•Spleen
•Liver
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Anatomy and Physiology of Spleen
• Small hand-sized organ in front of the left
kidney, behind the stomach
The largest lymphatic organ
Filters blood
Traps formed particles
Destroys bacteria
Serves as blood reservoir
Forms lymphocytes and monocytes
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Splenectomy
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Used to treat:
Idiopathic thrombocytopenic purpura
Immune system destroys platelets
ITP is sometimes called immune
thrombocytopenic purpura.
• ITP occurs when certain immune system cells
produce antibodies 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.
• Spleen out…platelets remain in the blood stream
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Splenectomy…
• Small percent develop an accessory
(extra) spleen…requiring removal
• More successful in younger <40
• >65 higher complications and fatality
rate
• More difficult time recovering from
pneumonia, hospital-based
infections, meningitis, etc.
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Splenectomy
•
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Introduction to Blood
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Blood Components
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Blood Components…
• Plasma – a straw colored liquid portion of the blood in
which the cells and platelets are suspended; makes
up approximately 50-55% of a blood consists of
water, amino acids, proteins, carbohydrate, lipids,
vitamins, hormones, electrolytes and cellular wastes
• Serum – is essentially the same as plasma only
without the fibrinogen and clotting factors
• The volume of blood is approximately 8% of total
body weight
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Blood Plasma
Makes up 55% of blood volume
Part of extracellular fluid (like ISF except 7% protein
instead of 2%)
Has 3 types of plasma proteins:
ALBUMIN - to increase osmotic pressure
GLOBULINS - to transport & for antibodies
FIBRINOGEN - basic for blood clotting
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Plasma
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Normal Types of Blood Cells
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RBC’s
Basophils (WBC’s)
Neutrophils (WBC’s)
Monocytes (WBC)
Eosinophils (WBC)
B- Lymphocytes
Platelet (Thrombocytes)
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Anatomy and Physiology
Erythrocytes: red blood cells (RBCs)
Erythropoietin stimulates production
O2 transport
Thrombocytes: platelets
Thrombopoietin stimulates production
Clotting
Leukocytes: white blood cells (WBCs)
Fight infection and antigens
Granular and agranular
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Stem Cell Family Pressure
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RBC’s
Biconcave, disc shaped cells also known as
erythrocytes
The production of red blood cells are called
erythropoiesis
Erythropoiesis is when RBC’s are being formed
within the bone marrow as erythroblasts, they mature
and turn into normoblasts and form reticulocytes and
they mature within the blood or spleen and become
erythrocytes
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Hematopoiesis
– Formation of blood cells
• Primary site is bone marrow
• RBCs - 175 billion per day
• Neutrophils - 70 billion per day
• Platelets - 175 billion per day
• Bone marrow will respond to inc. need with more production
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Hemoglobin
• Hemoglobin is contained within the red
blood cells and is the oxygen carrying
unit of red blood cells; it also carries
CO2 to the lungs
• The destruction of RBC’s is called
culling which live approximately 120
days
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The Path of a RBC
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Cellular Components of Blood
• ERYTHROCYTES - largest part; anuclear,
biconcave disks; designed to carry hemoglobin;
produced in red bone marrow; survive about 120
days
• PLATELETS – non-nucleated cell fragments; help
in homeostasis & coagulation; normall ct 150,000400,000; life span 10 days*
• LEUKOCYTES - help fight infection & mediate the
immune response
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Leukocytes – White Blood Cells (WBCs)
• NEUTROPHILS- first to arrive at site of
injury; phagocytosis; *55-70%; life 2-4hrs*
• EOSINOPHILS- regulate hypersensitivity
reactions; seen in allergic reactions *1-4%
• BASOPHILS- release heparin & histamine
from granules into tissue*0.5-1%
• MONOCYTES (Macrophages)phagocytosis; stimulate maturation of T
cells *2-8%; stimulate inflammatory process*
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Neutropenia
• Abnormal low count of neutrophils
• Adults: < 1700 / microliter of blood
• Children varies with age
VULNERABLE to infectious diseases
• Severe neutropenia
– < 500 cells per microliter of blood
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Lymphocytes
• ACTIVE IN IMMUNE RESPONSE
• COMPOSED OF:
B-cells - produce antibodies
T-cells - mediate immunologic
responses
• MAKES UP 20-40% OF WBCs
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Erythrocytes or
Red Blood Cells (RBCs)
• Size
– Denoted by “CYTIC”
• Normocytic
• Microcytic
• Macrocytic
• Density
– From amount of hemoglobin
– Denoted by “CHROMIC”
– Normochromic
– Hypochromic
– Hyperchromic
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Erythropoiesis
• Must have adequate space & perfusion
in the bone marrow
• Must have essential substrates like
IRON, FOLIC ACID, VITAMIN B12
• Erythropoietin from the kidney
stimulates it.
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Bone Marrow
• The bone marrow is the site of
hematopoiesis or blood cell formation.
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Bone Marrow Aspiration
• Taken from posterior iliac crest most
often
• Patient needs explanation & signed
consent form
• Local anesthesia used for skin & SQ but
not bone itself
• Sharp pain during actual aspiration
though brief
• Watch for bleeding & infection
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Bone Marrow Sites
• Sites for bone marrow aspiration
may include:
–Sternum
–Iliac Crest
–Tibia
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Bone Marrow Biopsy
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Video
• www.youtube.com
• Bone Marrow Aspiration & Biopsy
• 12-29-08 csmcd
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Frequently Used Laboratory Tests
in Hematology
CBC
RBC’s
Hgb
Hct
MCV
MCHC
Reticulocyte Count
WBC’s
Platelets
PT
INR
PTT
Fibrinogen
D- Dimer
Coomb’s Test (used for blood typing )use direct
Coombs
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Complete Blood Count
• RBC - RED BLOOD COUNT- actual count of
circulating RBCs in 1 mm of blood
M 4.5 – 6.0 ; F 4.0 – 5.5 million/mm3
• HCT - HEMATOCRIT - volume of packed
RBCs in 100 mls of blood; expressed as %;
nl M 40 – 50 %; F 37-47%
• HGB - HEMOGLOBIN - protein substance
that gives blood its color & is composed of
iron that is an oxygen carrier; nl M 1418g/dl; F 12-16g/dl
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Major Blood disorders and
Lab Findings
• Anemia – ↓ in Hgb & Hct
• Polycythemia – ↑ in Hgb. & Hct.
• Leukopenia & Neutropenia – ↓ in WBC
& neutrophil count
• Leukocytosis – ↑ in WBC
• Thrombocytosis – ↑ in platelet count
• Thrombocytopenia – ↓ in platelet count
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Reticulocyte Count
(Retic Count)
• Normal - 0.5%-1.5 % of total RBCs
• Measures the marrow’s production of
erythrocytes ability to respond to anemia & make
RBCs
• Increased:
– hemolytic anemia,
– hemorrhage
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Decreased:
pernicious
folic acid
iron def., Aplastic anemia
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Coagulation Studies
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Bleeding Time 1-9 min
Prothrombin Time (PT) 11 – 12.5 sec
Partial thromboplastin time (PTT) 60 – 70 sec
Activated Partial thromboplastin Time (APTT)
– quicker to perform 60 – 70 sec
• INR: 2.0 – 3.0 (1.5-2.5)
• Fibrinogen
• *** desired therapeutic ranges for
anticoagulant = 1.5 to 2.0 times normal values
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Erythrocyte Disorders
• Loss of O2 carrying capacity of the
blood due to low numbers or cells
that don’t function adequately
• Can categorize:
– Decreased production
– Increased destruction
– Blood loss
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What does Increased RBCs indicate??
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Defective RBC Production
• Iron Deficiency
• Vitamin B6 deficiency (megablastic)
• Decreased erythropoietin production
• Cancer/Inflammation
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Bleeding (from RBC) Loss
• Bleeding from the GI tract, Menorrhagia,
epistaxis
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Hemolytic
(From destruction of RBCs)
– Altered erythropoiesis
• Sickle Cell Anemia
• Thalassemia
– Autoimmune Anemia
– Drug Induced Anemia
– Mechanical Heart Valve Related Anemia
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Clinical Manifestations of Anemia
• Severity of the anemia
• Speed with which the anemia has
developed - more rapid > severity
• Duration of the anemia
• Metabolic requirements of the individual
- more active may be more symptomatic
• Other disorders or disabilities
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Physical Assessment Findings
• SKIN:
– pallor or icteric
– petechiae or ecchymosis
– tongue may be smooth
• CHEST:
– SOB or DOE
– tachycardia, irregular rhythm, murmurs &
gallops
– orthostatic hypotension,
• GI - may have large liver or spleen
• CNS - impaired cerebral or peripheral nerve
function; chronic def. B12 can cause irreversible
neurologic degeneration
50
Assessment with Anemia…
• Weakness, fatigue, malaise, pallor common
• May see jaundice with megaloblastic & hemolytic
anemia
• Tongue may be smooth & red with iron def. or
beefy red & sore with megaloblastic anemia
• Mouth corners may be ulcerated (angular cheilosis)
• Person may crave ice, starch, or dirt (pica)
• Nails may be brittle, ridged, or concave
• Nutritional assessment
• Cardiac status for tachycardia, palpitations, dyspnea,
dizziness, orthopnea,
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Signs & Symptoms of Anemia
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Assessment & Diagnoses
• GI system:
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nausea & vomiting & diarrhea
melena
anorexia
glossitis
• Menstrual periods and pregnancy
• Neuro Exam :
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emphasis on paresthesia
ataxia
poor coordination
confusion
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Assessment & Diagnoses…
• Activity intolerance RT weakness,
fatigue and malaise
• Altered nutrition RT inadequate intake of
essential nutrients
• Altered tissue perfusion RT inadequate
blood volume or hct
• Noncompliance with prescribed therapy
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Iron Deficiency Anemia
• RBCs contain decreased amounts of
hemoglobin due to decreased iron
supply
• RBCs are microcytic & hypochromic
• Can result from: blood loss, ↑body
demands, malabsorption, or dietary
deficits
• Peculiar S&S : glossitis, pagohagia,
stomatitis, cheilosis
• Treat with iron by mouth or injection
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Hemochromatosis
• Too much iron being absorbed from
the GI tract
• Primary: specific genetic problem
– Most common genetic disorder in US
– 1 of every 200 to 300 Americans
• Secondary: caused by diseases
– Thalassemia
– Sideroblastic anemia
– Large number of blood transfusions
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Hemochromatosis…
• Men more than women
• Caucasians of Western European
descent
• Men ages 30 -50
• Women ages > 50
• Can occur age 20
• Familiar
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Hemochromatosis…
• Symptoms
– Abdominal pain
– Fatigue
– Darkening of skin color (bronzing)
– Joint pain
– Loss of body hair
– Weight loss
– Liver and spleen swelling
Confirmed with liver biopsy
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Hemochromatosis…
• Treatment
– Phlebotomy:
• ½ liter of blood removed each week until the
body iron level is normal
• May take months/years to accomplish
• Specific diet to reduce iron from diet
• Prohibits alcohol
• Avoid iron cookware, raw seafood
Overtime: liver scarring/damage.
Extra iron builds up in other body tissues
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Iron Administration
• ORAL SUPPLEMENTS:
– stools may be green or black
– Take with ascorbic acid to inc. absorption
– May need to drink with straw to prevent staining of teeth
• BEST ABSORBED ON EMPTY STOMACH*
• Take with or after meals to avoid GI upset-not with
antacid
• Intramuscular injection –should use Z-track method
with needle change and 0.5ml of air to prevent
leakage of med
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Iron Supplement
• Take Iron on an empty stomach
because Iron absorption is reduced with
food especially dairy products
• Increase vitamin C to enhance
absorption
• Explain to pt that stool will be dark in
appearance
• Review Z track for Iron administration
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Z track
• Draw the medication up into the syringe using aseptic
technique
• Add 0.25 mL of air to the syringe
• Discard the needle used to draw up the medication
• Place a new needle 22 G – 2 – 3 inch long on the
syringe
• Make certain the needle is not in bright light
• Select the dorsal gluteal ONLY
• Once the site is selected, pull the sq tissue sideways
away from the muscle (z track)
• Cleanse the site and insert into the muscle tissue
• Aspirate to determine placement
• If blood is aspirated, discard and begin again
• Inject the medication slowly
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Iron Sources
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Organ meats - liver, red meats
Kidney beans
Iron-fortified cereals
Dark, green leafy vegetables, legumes
Whole grains
Blackstrap molasses
Dried fruit (raisins)
Foods cooked in iron pans
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Nursing Interventions
• Managing fatigue
• Maintaining adequate nutrition - well
balanced diet
• Maintaining adequate perfusion acute blood loss may require IV
replacement with fluids or
transfusions, oxygen & close
monitoring - NOTE TACHYCARDIA
• Complying with prescribed therapy understand medication regimen
• Monitor for complications - CHF &
neuro
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Folic Acid Deficiency Anemia
• Causes megaloblastic anemia
• Similar to B12 deficiency without the
nervous system manifestations
• Caused by: Poor nutrition (esp.
lacking leafy green veg., liver, citrus
fruits, dried beans, nuts) as seen in
alcoholics Malabsorption syndromes
like Crohn’s . Drugs that inhibit folic
acid metabolism like methotrexate,
anticonvulsants, & oral
contraceptives
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Folic Acid Foods
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Liver
Organ Meats
Eggs
Cabbage
Broccoli
Brussels sprouts
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Vitamin B12 Deficiency Anemia
• PERNICIOUS ANEMIA- caused by a deficiency of
adequate intrinsic factor (IF) which is essential for
the intestinal absorption of vitamin B12
• Macrocytic RBCs ; do Schilling test
• S&S: smooth sore tongue, numbness & tingling
• of hands & feet
• Tx with B12 (cyanocobalamin) injections IM
• OTHER CAUSES- inadequate dietary intake or
poor absorption of vitamin B12
• Vitamin B12 foods: milk, eggs, meat, liver, OJ,
spinach
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Assess tongue carefully for different types of anemia’s
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B12 foods
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Liver
Organ Meats
Dried Beans
Nuts
Green Leafy Vegetables
Citrus Fruits
Brewer’s Yeast
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Nursing Assessment:
Vitamin B¹² Deficiency
Chief complaint and past medical history
Family history
Review of systems
Functional assessment
Physical examination
Signs of hematologic abnormalities:
– Petechiae
– Purpura
– Ecchymosis
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Anemias of Renal Disease
• End stage renal disease:
– shorten RBC life
– cause a deficiency in erythropoietin
• Dialysis patients:
– lose blood in dialyzer & become iron
deficient
– possibly folic acid deficient
• May need to take recombinant
erythropoietin (Epogen) & oral iron
supplements
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Anemia of Chronic Diseases
• Extremely common
• RBCs may look normal
• TIBC ↓, Fe ↓
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infections
cancer
inflammatory disease
malnourished state
• Not understood but does not respond
to Fe
• Treatment: packed RBCs
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Aplastic Anemia
• Results from a deficiency of circulating
erythrocytes resulting from arrested
development of RBC’s within the bone
marrow
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Aplastic Anemia
• Common causes:
– Exposure to myelotoxic agents
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Radiation
Benzene
Alkylating agents
Antimetabolites
Sulfonamides
Insecticides
– Viral infection (unproven)
• Epstein-Barr virus
• Hepatitis B
• Cytomegalovirus
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Aplastic Anemia
• Caused by bone marrow malfunction
• Three problems together:
– Erythrocytopenia,
– Leukopenia
– Thrombocytopenia
• May present with:
– Fatigue
– pallor,
– DOE
• Treatment of choice is bone marrow transplant
replaces the defective stem cells or removal of spleen
that may be destroying normal RBCs or suppressing
their development
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Megaloblastic anemia
• Characterized by macrocytic red cells
• Main problem caused from defective
DNA synthesis found in vitamin B12 or
folic acid deficiency
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Megaloblastic anemia…
• Deficiency of Vitamin B12
– Poor intake of foods containing B12
• Vegetarian diets
• Diets lacking dairy products
• Problems of small intestine
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Small bowel resection
Diverticulitis
Tapeworm
Overgrowth of intestinal bacteria leads to poor
absorption of B12
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Hemolytic Anemia
• May be Inherited or Acquired
• 200 disorders are known
• Due to increased RBC destruction
missing enzymes
• RBC survival may be < 15-20 days
• I. Sickle Cell Anemia
• II. G-6-PD Deficiency Anemia
III. Autoimmune Hemolytic Anemia
• IV. Extrinsic Factors
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Inherited Hemolytic Anemia
• Sickle Cell
• Thalassemia
• G-6-PD
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Acquired Hemolytic Anemia
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Liver disease
Uremia
Trauma
Mechanical Heart Valve
Infection
DIC
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Blood Loss Anemia
• ACUTE : trauma to blood vessels or internal
hemorrhage
• Degree may be hard to tell since lowest hct may
not be reached till 24hrs later & retic ct does not
rise for 3-5day
• May see low BP, inc. HR, weakness, cool skin & light
headed
• CHRONIC : gastrointestinal & menstrual bleeding
• Associated with Fe deficiency so microcytic &
hypochromic
• ID source to stop & treatment by iron supplements
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Sickle Cell anemia
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Sickle Cell Anemia
• Inherited autosomal recessive disorder of
hemoglobin
• Primarily persons of African an Eastern
Mediterranean descent
• Appears after 4 months old
• Hemoglobin S (HgbS) replaces all or part of
normal hemoglobin, which causes the RBCs
to sickle when oxygen is released into the
tissues
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Sickle Cell Anemia…
• HgbS less than normal lifespan: 40 days
• Leads to chronic anemia
• Crisis is characterized by severe pain
that occurs when blood flow is
obstructed
• Treatment: hydration, oxygen, pain
medication, and administration of
Hydroxyurea
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Pregnancy…
• Autosomal recessive inheritance
• Both parents must pass the defective
form of the gene for a child to be
affected
• If only one parent passes the gene,
that child will have the sickle cell trait
• Two people with sickle cell trait:
– 25% having unaffected child
– 50% having a child who is a carrier
– 25% having a child with sickle cell
anemia
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Clinical Manifestations of Sickle Cell Anemia
• Vaso-occlusive crisis: the classic sign
– Severe abdominal pain
– Fever
– Painful edematous hands & feet
– Arthralgia
– Leg ulcers (adolescents)
– Delayed growth
– Frequent infections
– Anemia
– CVA (↑ risk with dehydration)
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Complications of Sickle Cell
Anemia
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Stroke
Acute chest syndrome
Pulmonary hypertension
Organ damage
Blindness
Skin ulcers
Gallstones
Priapism
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Care of the Patient in sickle Cell Crisis
• Oxygen therapy
• Drug therapy –48 hours of IV analgesics
• Hydrate the patient with normal saline
and oral fluids without caffeine
• Transfusion therapy
• Do not raise knee gatch of the bed
• Keep room temperature at or above 72
degrees
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Drug therapy
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Morphine & hydromorphine(Dilaudid)
Opioid addiction rare
Avoid IM (absorption impaired)
Moderate pain
– NSAIDs
– Complementary therapies
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Warm room
Distraction
Relaxation
Aroma therapy
Therapeutic touch
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Drug Therapy…
• Hydroxyurea is a chemotherapy agent
that has been shown to increase
hemoglobin F levels thereby decreasing
the permanent formation of sickled cells
– Suppresses bone marrow function
– Increased incidences of leukemia
– Drug toxicity
– Causes birth defects
• Folic Acid Replacement
• Treat infection promptly with Antibiotics
(Haemophilus Influenzae)
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Treatments….
• Bone marrow transplant only
potential cure: hard to find donor
• Blood transfusion: excess amount of
iron
– Deferasirox (Exjade)
• Heavy metal chelating agent
• Stem cell transplant
– Long hospital stay
– Life-threatening complications
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Experimental treatments
• Gene therapy
– Insert normal gene into bone marrow for
normal hemoglobin
– Turning off defective gene while
reactivating another gene that prevents
sickle cells from forming
• Nitric oxide
– Prevent sickle cells from clumping
together
• Drugs to boost fetal hemoglobin
production
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Women’s Health Considerations
• Pregnancy in women with sickle cell
disease may be life threatening. Patients
who have damage to vital organs are
advised against becoming pregnant. The
use of oral contraceptives is controversial,
because Ocs may increase clot formation,
especially among smokers, predisposing
them to crises. The risks versus benefits
must be examined for each patient
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Thalassemia
• Group of hereditary disorders associated with
defective hemoglobin chain synthesis
• Found in people of Mediterranean, Africa and
Southeast Asian
• Characterized by Hypochromia (abnormal
decrease in the Hgb of RBC’s) and extreme
microcytosis (smaller than normal RBC’s) and
destruction of blood
• Males and females
• Lifespan: 20-30 years
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Thalassemia…
• There are two types:
– Alpha (microytic )
• Stillborn
• Bone deformities of face
• Growth failure
– Beta (can be fatal)…Cooley’s Anemia –
Chelation therapy is used (too much iron
that is removed after chronic transfusions)
• Normal at birth develops severe anemia during
1st year of life
– Blood transfusion/ folate supplements
– Bone marrow transplant helps children
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Alpha thalassemia (Hemoglobin Bart's)
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Glucose-6-Phosphate
Dehyrogenase Deficiency
• Hereditary condition in which RBCs break
down when body is exposed to certain
drugs/stress of infection
• Most common human enzyme deficit
• “Favism”: allergic to fava beans
• Common black males
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G-6-PD…
• Affected / episode triggered by certain
medications:
– ASA
– Antimalarial
– Nitrofurantoin
– NSAIDS
– Sulfa
– Common: mothballs
• Spontaneous recovery from a hemolytic episode
is the usual outcome
• Prevention: avoid triggers
• **Benefit: confers a resistance to malaria**
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Hereditary Spherocytosis
• Type of hemolytic anemia
• Characterized by abnormal permeability
of the RBC membrane
• Causes spherical shape and causes
RBC’s to be destroyed prematurely and
sent to the spleen
• Spleen usually has to be removed
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Red Blood Cell Disorders
• Polycythemia Vera: production of too
many red blood cells
• Anemias: deficiency of red blood cells or
hemoglobin caused by
– Blood loss
– Iron deficient diet
– Vitamin B12 deficiency (pernicious anemia)
– Bone marrow failure (hemolytic anemia)
– Genetic abnormalities
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Polycythemia Vera
• Dark color of the skin
104
Polycythemia Vera –Cancer of RBCs
• Malignant disease
• Disease with a sustained increase in
hemoglobin or hematocrit
• Massive production of RBCs…hallmark
• Excessive leukocyte production…hallmark
• Excessive production of platelets…hallmark
• Plethoric (dark, facial appearance from the
blood is so oxygenated
• Medical Treatment – If untreated may die
within 2 yrs
• Men > women; rare < 40 years of age
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Symptoms
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Breathing difficulty when lying down
Dizziness
Excessive bleeding
Fullness in the left upper abdomen (due to
enlarged spleen)
Headache
Itchiness, especially after a warm bath
Red coloring, especially of the face
Shortness of breath
Symptoms of phlebitis
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Polycythemia Vera Treatment
• Phlebotomies (2-5 times/week)
• Therapy aimed to prevent clot
formation
– Aspirin
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Treatment
• Reduce the thickness of the blood and prevent
bleeding and clotting.
•
Phlebotomy is used to decrease blood thickness.
One unit of blood (about 1 pint) is removed weekly
until the hematocrit level is less than 45 (males) or 42
(females). Then therapy is continued as needed.
• Chemotherapy (specifically Hydroxyurea) may be
given to reduce the number of red blood cells made
by the bone marrow. Interferon may also be given to
lower blood counts. A medicine called anagrelide may
be given to lower platelet counts.
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Patient Education /guide for Polycythemia
Vera
• Drink at least 3 Liters of liquids a day
• Avoid tight or constrictive clothing
• Wear gloves when outdoors in
temperatures lower than 50 degrees F
• Take anticoagulant as ordered
• Wear support hose
• Elevate feet whenever possible
• Use electric razors
• Use a soft bristled toothbrush
• Do not floss between teeth
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77-year old female
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WBC and Immune Disorders
• Immunocompromised patient
– Defect in WBC or immune physiology
111
Immunocompromise
•
•
•
•
Defects in WBCs or immune physiology
Lack of normal defenses
May be asymptomatic
Infection is leading cause of death
112
Immunocompromise…
• Symptoms of infection often absent
• Fever may be only sign of infection
• Pain without signs of inflammation is
also a cue
• Anergy may be noted with the use of
skin tests
113
Laboratory Analysis
•
•
•
•
Leukopenia
Low CD4 counts
Decreased immunoglobulins
No response to antigen skin test
114
Causes of Leukopenia
• Primary cause:
–
–
–
–
–
–
–
–
–
–
extensive neutrophil use
reduction in the number of neutrophils.
chemotherapy
radiation therapy
leukemia
Myelofibrosis
aplastic anemia, influenza
Hodgkin's lymphoma
Folate deficiencies, psittacosis and sepsis
Other causes are deficiency in certain minerals,
such as copper and zinc.
115
Medical Management
• Reverse the cause
– Cell replacement
– Bone marrow transplant
– Immunoglobulins
• Treat infections
• Nutrition
116
Nursing Management
•
•
•
•
•
•
Isolation
Hygiene
Aseptic technique for procedures
Good assessment for cues of infection
Maintain skin integrity
Optimum nutritional support
117
Neutropenia
• Absolute neutrophil count less than
1000
• Causes
– Inadequate production
– Excessive destruction
• High risk of infection
118
Neutropenia Risks
• Overwhelming infection
• Radiation therapy
• Chemicals and drugs
– Antibiotics
– Chemotherapy
• Disease states
119
Assessment of Neutropenia
• Classic symptoms of infection often
absent
• Areas of heavy bacteria at risk for
infection (mouth, perineum, IV sites,
catheter)
120
121
122
Neutropenia:
Medical Management
•
•
•
•
Prevent and treat infection
Reverse cause
Colony-stimulating factors
Prophylactic antibiotics
123
Neutropenia:
Nursing Management
• Similar to that used for
immunocompromised patient
124
Malignant WBC Disorders
Leukemia
Lymphoma
Multiple myeloma
125
Leukemia
• Malignant neoplasm of the blood-forming
organs
• Abnormal over production of immature forms
of any of the leukocytes
• Interference with normal blood production that
results on decreased number of RBCs and
platelets
126
Leukemia…
• Exact cause: unknown
• Precipitating factors:
– Highest in children 3 to 4 years
– Declines until age 35 then steady ↑
127
Assessment Findings
•
•
•
•
•
•
Petechiae
Ecchymosis
Enlarged Lymph nodes
Joint pain
Hematemesis
Diagnostics show decreased RBC’s and
platelets, immature WBC’s and bleeding
time
• Bone marrow biopsy shows large
number of immature leukocytes
128
Leukemia…
• Acute leukemia
– Bone marrow cells cannot mature properly
– Immature cells continue to reproduce and
build up
• Chronic leukemia
– Cells can mature partly-not completely
– These cells are not normal
– Survive longer
– But cells continue to build up and crowd out
normal cells
129
Medical Management
• Radiation
• Chemotherapy
• Transfusion therapy of platelets, packed
RBC’s whole blood
• Antipyretics
• Analgesics
130
Leukemia Interventions
•
•
•
•
•
•
Infection: major cause of death
Sepsis common complication
Auto-contamination
Cross-contamination
Drug therapy
Hematopoietic stem cell transplantation
131
Review
• Page 903
–Chart 42-6
–Need to know the
differences
132
Acute Myeloid Leukemia (AML)
• Group of disorders characterized by
replacement of the bone marrow with
abnormal immature cells of myeloid
• Nonspecific symptoms:
– Anorexia
– Malaise
– Irritability
– Weight loss
– Fever
– Bone pain
133
AML…
• Most common adults > 65 years
• Males > female
• Previous childhood ALL
• Causes:
– DNA mutations
– Radiation, exposure to certain chemicals
and some chemotherapy drugs may play a
role.
134
Acute Myeloid Leukemia (AML)…
• Treatment
–Bone marrow transplant
–Chemotherapy
–50% cured/remission
135
Acute Myeloid Leukemia (AML)…
136
Chronic Myelogenous Leukemia
• Rare in children
• Uncommon in people < 20 years
• Philadelphia Chromosome found in 9095%
• Chromosomes switch (9 & 22) and
combine creating a new gene:
BCR/ABL
(which is found in virtually all patients with
CML)
137
CML…
• Mostly males
• Causes:
– High levels radiation
– Atomic bomb survivors
• Symptoms:
– Tiredness
– SOB with activities
– Pale looking skin
– Enlarged spleen
– Night sweats
– Weight loss
138
CML…
• 3 phases:
– Chronic
• Slowing progressing
– Accelerated phase
• Spleen enlargement
• 6-12 months
– Blast phase
• Aggressive acute leukemia
Are not cured! Remission long periods
139
Acute Lymphocytic Leukemia (ALL)
• Malignant disease of bone marrow
• Most common Leukemia in children
• Physical signs:
– Pallor
– Cardiac flow murmur
– Fever/signs of infection
– Petechiae of LE’s
– Rashes from infiltration of the skin with
leukemic cells
– Prognosis: 80% of children >years or longer
140
Chronic Lymphocytic Leukemia (CLL)
• Cancer of blood and bone marrow
• Progression slow/affects WBC’s
• Rare symptoms in early phase
– Enlarged but painless lymph nodes
– Fever
– weight loss/loss of appetite
– Lethargy/fatigue
– Night sweats
– Pain/fullness below ribs left side
141
Chronic Lymphocytic Leukemia (CLL)…
• Causes: damage to the DNA of
developing cells in the bone marrow
• Researchers: discovered people with
CLL have chromosome deletions.
• Affect older adults ≥ 60 /Caucasians
• Not inherited/few risk factors
– Herbicides
– insecticides
142
Hodgkin Disease
•
•
•
•
•
Malignancy of the lymphoid system
Painless Lymphadenopathy
Cause unknown
Prognosis good: 5 year 90%
Diagnosis: excision of node for biopsy
– Reed-Sternberg cells
143
Hodgkins Disease…
• More common in men and has two
peaks 15-35 years of age and 55-75
years of age; incidence is higher in
Caucasians than in African Americans
• The cause of the disease is unknown
although several factors have been
identified to contribute to the
development of the disease including
Epstein Barr virus (EBV) and exposure
to toxins
144
Hodgkin's…
•
•
•
•
•
•
1% of all cancers USA
20-30 years of age….50 or greater
6th most common cancer in USA males
5th in woman
Steadily increasing
Actually a group of >30 types of cancers
145
Staging of disease
• Stage l
– Single lymph node region/site
• Stage ll
– Two or more lymph nodes on same side of
the diaphragm or site
• Stage lll
– Lymph node areas on both sides of the
diaphragm to one extralymphatic organ, the
spleen, or both
• Stage lV
– Diffuse one or more extralymphatic organ,
with or without lymph node involvement
146
Non-Hodgkins Lymphoma
• Non Hodgkin's Lymphoma is type of
cancer than originates from lymphoid
tissue that are not diagnosed as
Hodgkin's Lymphoma
• Treatment:
– chemotherapy
– radiation
– multiagent chemotherapy
147
Non-Hodgkins Lymphoma…
• Symptoms:
– Swollen lymph nodes in your neck, armpit
or groin
– Abdominal pain or swelling
– Chest pain, coughing or trouble breathing
– Fatigue
– Fever
– Night sweats
– Weight loss
148
Multiple Myeloma
• Multiple myeloma: cancer of your plasma cells, a type
of white blood cell present in bone marrow.
• Plasma cells normally make proteins called antibodies
to help fight infections.
• In multiple myeloma, a group of plasma cells
(myeloma cells) becomes cancerous and multiplies,
raising the number of plasma cells to a higher than
normal level.
• Since these cells normally make proteins
(antibodies), the level of abnormal proteins in your
blood also may go up.
• Health problems caused by multiple myeloma can
affect your bones, immune system, kidneys and red
blood cell count.
149
Multiple Myeloma…
•
•
•
•
•
•
Rare in people younger than 60
High American Black Males
Incurable
“Spike” pattern of plasma proteins
IgG, IgA
Treatment varies
150
Multiple Myeloma…
• 4 Common Major Problems
– Ca++ high levels
– Kidney failure
– Anemia-related fatigue
– Bone damage and fracture
CRAB
151
Multiple Myeloma…
• Symptoms:
– Common bone pain
•
•
•
•
Back
Pelvis
Ribs
Skull
– Repeated infections
– obesity
152
Thrombocytopenia
• Decreased platelets
– <100,000µl
• Risk for bleeding
• Treated with platelet transfusion
153
Care…Thrombocytopenia
•
•
•
•
•
•
•
•
•
•
Handle the patient gently
Use a sheet to life patient
Avoid IM injections
Apply firm pressure to all sticks
Do not perform rectal temps, give enema’s &
tell to avoid intercourse
Teach to use electric razors
Soft bristled tooth brushed
Avoid dental work
Encourage not to blow nose
Check all urine and stool for blood
154
Thrombocytopenia…
155
Idiopathic Thrombocytopenic Purpura (ITP)
•
•
•
•
•
•
Low number of platelets
Cause unknown
Purpura : skin or mucous membrane
Petechiae: red/purple dots
Nosebleeds, gums bleed, heavy menses
? Immune system might be the cause:
your immune system attacks and
destroys its own platelets: for an
unknown reason
156
Idiopathic Thrombocytopenic Purpura (ITP)…
• Risk factors:
– Women 2X > males
– Age: common > 60 years
– Recent viral infection
• Bleeding rare with ITP
• Complications arise from treatment
– Corticosteroids/surgery
Children: follows viral (mumps/flu)
Clears on its own within 2-8 weeks
with or without treatment
157
Thrombotic Thrombocytopenic Purpura
• TTP
• Rare disorder: platelets clump together
abnormally in the capillaries & too few
platelets remain in circulation
• Inappropriate clotting yet blood fails to
clot properly when trauma occurs
• Treatment: plasmaphersis, fresh frozen
plasma, aspirin, immunosuppressive
therapy
158
Vitamin K deficiencies
• Proper liver function and bile
production are critical for
formation of vitamin K
159
Hyperhomocystinemia
• Homocysteine – causes endothelial
dysfunction…leads to smooth muscle growth
= atherosclerosis
• Inherited/acquired early stage of renal
failure/renal transplantation/vitamin B12
deficiency
• High levels = risk factor for ischemic stroke
• Hyperhomocystinemia =
Independent risk factor for vascular disease
160
Bleeding Disorders
• Abnormality in stages of clotting
– Vasoconstriction
– Creation of platelet plug
– Development of clot
– Fibrinolysis
• Inherited or acquired
• Common in renal, hepatic, and
gastrointestinal disorders; malnutrition
161
Tests Measuring Bleeding and
Coagulation
• CBC
– Hemoglobin
– Hematocrit
• Fibrinogen
• Prothrombin time
• International normalized ratio
• Partial thromboplastin time
• Platelet agglutination/aggregation
162
Nursing Diagnoses
•
•
•
•
•
Risk for bleeding
Pain
Deficient fluid volume
Altered self-image
Altered tissue perfusion
163
Coagulation Disorders
• Thrombocytopenia
– Idiopathic (ITP)
– Thrombotic Thrombocytopenic Purpura
(TTP)
• Hemophilia
– Genetic disorder
– Absence of Factor VIII
– Treated with replacement of missing
clotting factor
164
Von Willebrands Disease
• The disease is caused by a deficiency of
factor VII
165
Hemophilia
• Hemophilia comprises two hereditary bleeding
disorders resulting from deficiencies of
specific clotting factors
• Hemophilia A results from a deficiency of
factor VIII
• Hemophilia B (Christmas Disease) is a
deficiency of factor IX
• Hemophilia is an X recessive trait
• Hemophilia A is a disease affecting males,
non of whose sons will have the gene for
Hemophilia and all whose daughters will be
carriers
166
Medical Management: Bleeding
•
•
•
•
•
•
•
•
•
Whole blood
Packed RBCs
Leukocyte-poor RBCs
Platelets
Cryoprecipitate
Albumin
Granulocytes
Plasma protein
Fresh frozen plasma
167
DIC
• DIC is a syndrome characterized by
abnormal initiation and acceleration of
clotting and simultaneous hemorrhage
• Risk Factors for DIC include venomous
snakebite, sepsis, trauma, obstetric
complications, hypoxia, tissue necrosis,
drug reaction, extensive burns,
prosthetic devices
168
Pathophysiology of DIC
• Initiating event: procoagulants
• Stimulation of intrinsic or extrinsic
pathway
• Clots in microvasculature
• Consumption of clotting factors
• Fibrinolysis
• FDPs: potent anticoagulants
169
Etiology of DIC
• Infection
• Trauma (e.g., burns, crush)
• Obstetric conditions (e.g., Abruptio,
amniotic fluid embolus, retained dead
fetus)
• Hematologic disorders
• Oncologic disorders
• Other: shock or sepsis, acute respiratory
distress syndrome
170
Assessment of DIC
• Evidence of occult bleeding
• Signs of platelet deficiency
– Petechiae
– Ecchymosis
171
Clinical Manifestations of DIC
• Integumentary – pallor, purpura, ecchymosis,
hematomas, gingival bleeding, bleeding from
puncture sites
• GI – Hemoptysis, Melena, Occult Blood,
Abdominal Distention, abdominal pain
• Respiratory – dyspnea, tachypnea, orthopnea,
decreased breath sounds, CP
• Cardiac – decreased pulses, tachycardia,
venous distention
• Genitourinary – hematuria,
172
Laboratory Diagnosis: DIC
•
•
•
•
•
•
•
Decreased platelets
Decreased fibrinogen
Prolonged PT, aPTT, thrombin time
Elevated FDP or FSP
Increased D-dimer
Decrease in coagulating factors
Decrease in hemoglobin and hematocrit
173
Treatment of DIC
• Correct underlying cause
• Administer blood and components
– Platelets
– Fresh frozen plasma
– Cryoprecipitate
– Packed RBCs
• Stop abnormal coagulation
– Heparin: controversial when experiencing
more hemorrhage than thrombosis
174
Nursing Management of DIC
•
•
•
•
•
•
Assess and prevent
Frequent laboratory analysis
Administration of blood products
Assess circulation
Relieve pain
Assess for complications: shock,
multisystem organ failure
175
Transfusion Therapy
•
•
•
•
•
•
•
•
•
Assess lab values
Verify MD orders
Assess baseline vital signs
Need IV access with #20 Jelco or greater
Obtain blood product from blood bank
Check with two RN’s
Use only normal saline because of hemolysis
Blood must be hung within 15 minutes
CANNOT hang longer than 4 hours because of
bacterial growth
• Remain with patient during first 15-30 minutes of
infusion
• Administer blood using the appropriate filter
176
177
Possible Diseases from
Transfusions
•
•
•
•
•
Hepatitis B, C
AIDS
CMV (Cytomegalovirus)
Graft-Verus-Host Disease
Crutzfeldt-Jacob Disease (fatal)
– Mad Cow Disease
– is a rare, degenerative, invariably fatal brain
disorder
– Unknown, inherited or disease is
transmitted by exposure to brain or nervous
system tissue
178
Platelet Transfusions
• Platelets are administered to patients
with platelet counts below 20,000 mm
and to patients with thrombocytopenia
• Platelets are administered
• rapidly since they are fragile
• Standard sets are not used
179
Plasma Transfusion
• Freezing preserves the clotting factor
• FFP – or fresh frozen plasma is infused
immediately after thawing while the
clotting factors are still viable
• ABO compatibility is required for
transfusion of plasma
180
Cryoprecipitate
• Cryoprecipitate is a product derived from
plasma
• Clotting factors VIII von Willebrands
factor, fibrinectin, and fibrinogen are
precipitated from pooled
plasma to produce
cryoprecipitate
181
Transfusion Reactions
• Can occur when whole blood, PRBCs,
platelets, or FFP are given
• 4 types of reactions can occur:
– Hemolytic
– Anaphylactic
– Febrile
– Circulatory overload
182
Hemolytic Transfusion Reaction
• Caused by blood type or RH
incompatibility
• These complexes can destroy the
transfused cells and initiate
inflammatory responses in the recipients
blood vessel walls and organs
• S/S include apprehension, HA, CP, low
back pain, tachycardia, tachypnea,
hypotension, hemoglobinuria
183
Allergic Transfusion Reaction
• Allergic reaction are most often seen in
patients with a history of allergy
• They may have urticaria, itching,
bronchospasms or occasionally
anaphylaxis
• Onset usually occurs within 24 hours
after transfusion
• Can receive “buffy coat” or washed cell
184
Febrile Transfusion Reaction
• Febrile Transfusion reaction occur most
commonly in the patient with anti-WBC
antibodies, a situation seen after
multiple transfusions
• S/S – sensation of cold, tachycardia,
fever, hypotension, tachypnea
• Leukocyte filters may also be used to
trap WBC’s
185
Bacterial Transfusion Reaction
• Bacterial transfusion reaction are seen
after transfusion of contaminated blood
• Usually a gram negative organism
• S/S – fever, chills, shock, tachycardia,
hypotension
186
Circulatory Overload
• Circulatory overload can occur when a
blood product is administered too
quickly
• Usually associated with whole blood
• S/S – hypertension, bounding pulse,
distended jugular veins, restlessness,
confusion
187
Transfusion-Associated GraftVersus-Host Disease
• Seen in patients who are
immunocompromised
• Involved the donor attacking the host
tissues
188