Download Module 1apresentation

Module 1
HEMATOLOGIC/IMMUNE/AUTOIMMUNE
W.PAWLIUK MPH MSNED RN CEN
COMPOSITION OF BLOOD
 Blood
 Composed of plasma and formed elements
 Complex transport medium that performs vital pickup and
delivery services for the body
 Keystone of body’s heat-regulating mechanism
 Blood volume
 Blood volume varies according to age, body type, sex, and
method of measurement
FORMED ELEMENTS OF BLOOD
 Red blood cells (RBCs), also called erythrocytes


Description of mature RBCs
 Have no nucleus and shaped like tiny biconcave disks
 Do not contain ribosomes, mitochondria, or other organelles typical of
most body cells
 Primary component is hemoglobin
 Most numerous of the formed elements
Function of RBCs
 Critical role in the transport of oxygen and carbon dioxide depends on
hemoglobin
FORMED ELEMENTS OF BLOOD (cont.)

Hemoglobin
Within each RBC are approximately 200 to 300 million molecules
of hemoglobin
 Hemoglobin is composed of four globin chains, each attached to a
heme group
 Hemoglobin is able to unite with four oxygen molecules to form
oxyhemoglobin to allow RBCs to transport oxygen where it is
needed
 Anemia: a decrease in number or volume of functional RBCs in a
given unit of whole blood

FORMED ELEMENTS OF BLOOD (cont.)

Formation of RBCs
Erythropoiesis: entire process of RBC formation
 Formation begins in the red bone marrow as hematopoietic stem
cells and goes through several stages of development to become
erythrocytes; entire maturation process requires approximately 4
days
 RBCs are created and destroyed at approximately 100 million per
minute in an adult; homeostatic mechanisms operate to balance
the number of cells formed against the number of cells destroyed

FORMED ELEMENTS OF BLOOD (cont.)

Destruction of RBCs (Figure 17-8)
Life span of a circulating RBC averages 105 to 120 days
 Macrophage cells phagocytose the aged, abnormal, or fragmented
RBCs
 Hemoglobin is broken down and amino acids, iron, and bilirubin
are released

FORMED ELEMENTS OF BLOOD (cont.)
 White blood cells (WBCs), also called leukocytes (Table 17-1)

Granulocytes
 Neutrophils: approximately 65% of total WBC count in a normal blood
sample; highly mobile and active phagocytic cells

Eosinophils: 2% to 5% of circulating WBCs; numerous in lining of
respiratory and digestive tracts; capable of ingesting inflammatory
chemicals and proteins associated with antigen-antibody reaction
complexes; provide protection against infections caused by parasitic
worms and allergic reactions

Basophils: only 0.5% to 1% of circulating WBCs; motile and capable of
diapedesis; cytoplasmic granules contain histamine and heparin
FORMED ELEMENTS OF BLOOD (cont.)

Agranulocytes
Lymphocytes: smallest of the WBCs; second most numerous
WBC; account for approximately 25% of circulating WBCs; T
lymphocytes and B lymphocytes have an important role in
immunity: T lymphocytes directly attack an infected or
cancerous cell, and B lymphocytes produce antibodies
against specific antigens
 Monocytes: largest leukocytes; mobile and highly phagocytic
cells


WBC numbers: normal blood usually contains 5000 to
9000 leukocytes, with different percentages for each type;
WBC numbers have clinical significance because they
change with certain abnormal conditions
FORMED ELEMENTS OF BLOOD (cont.)

Formation of WBCs

Granular and agranular leukocytes mature from the
undifferentiated hematopoietic stem cell

Neutrophils, eosinophils, basophils, and a few lymphocytes and
monocytes originate in red bone marrow; most lymphocytes and
monocytes develop from hematopoietic stem cells in lymphatic
tissue
BLOOD TYPES: BLOOD GROUPS
 The ABO system

Every person’s blood belongs to one of four ABO blood groups

Named for antigens on RBC membranes
Type A: antigen A on RBCs
 Type B: antigen B on RBCs
 Type AB: both antigens A and B on RBCs; known as universal
recipient
 Type O: neither antigen A nor B on RBCs; known as universal
donor

BLOOD TYPES: BLOOD GROUPS (cont.)
 The Rh system

Rh-positive: Rh antigen is present on the RBCs

Rh-negative: RBCs have no Rh antigen present

Anti-Rh antibodies are not normally present in blood; anti-Rh
antibodies can appear in Rh-negative blood if it has come in
contact with Rh-positive RBCs
Anemia
 Reduction in the total number of
erythrocytes in the circulating blood or in the
quality or quantity of hemoglobin




Impaired erythrocyte production
Acute or chronic blood loss
Increased erythrocyte destruction
Combination of the above
Anemia
 Classifications
 Morphology
Based on MCV, MCH, and MCHC values
 Size
 Identified by terms that end in “-cytic”
 Macrocytic, microcytic, normocytic
 Hemoglobin content
 Identified by terms that end in “-chromic”
 Normochromic and hypochromic

Anemia
 Physiologic manifestation
 Reduced oxygen-carrying capacity
 Variable symptoms based on severity and the
ability for the body to compensate
Anemia
 Risk factors
 Advanced age, environmental exposure to chemicals, liver
disorders, autoimmune or other immunological disorders
 Chronic anemia symptoms
 Fatigue, weight loss, pallor, dyspnea on exertion,
hematochezia, sensitivity to cold, intermittent dizziness,
excessive menstration, paresthesias, uremia weakness, melena
 Acute anemic symptoms
 Fever, chest pain, acute heart failure, confusion, irritability
orthostatic hypotension, dyspnea, tachypnea, frank bleeding
Anemia
 Pernicious anemia
 Caused by a lack of intrinsic factor from the gastric
parietal cells
 Required for vitamin B12 absorption
 Results in vitamin B12 deficiency
Anemia
 Pernicious anemia
 Typical anemia symptoms
 Neurologic manifestations


Absence of intrinsic factor on testing
Others

Loss of appetite, abdominal pain, beefy red tongue (atrophic
glossitis), icterus, and splenic enlargement
Anemia
 Pernicious anemia
 Treatment

Parenteral or high oral doses of vitamin B12
Anemia
 Aplastic anemia
 Pancytopenia
 Pure red cell aplasia
Anemia’s compared
Pernicious
Aplastic
 Lack of intrinsic factor
 Rare, caused by bone
for absorption of B12



Could be congenital or
adult-onset gastric
mucosal atrophy
Develops slowly and as
hemoglobin decreases to
around 7-8 g/dl classic
symptoms start appearing
Treatment: Vitamin B12
marrow problems,
autoimmune disorders,
may also be congenital


Classic symptoms seen
Treatment is targeted at
the cause but can include
transfusions
Hemolytic Anemia
 Destruction or hemolysis of RBCs at a rate that
exceeds production
 Third major cause of anemia
 Intrinsic hemolytic anemia



Abnormal hemoglobin
Enzyme deficiencies
RBC membrane abnormalities
Hemolytic Anemia (Cont’d)
 Extrinsic hemolytic anemia
 Acquired
 Sites of hemolysis
 Intravascular
 Extravascular
Sequences of Events in Hemolysis
Hemolytic Anemia
 Jaundice
 Destroyed RBCs cause increased bilirubin
 Enlarged spleen and liver
 Hyperactive with macrophage phagocytosis of the defective
RBCs
Sickle Cell Disease (SCD)
 Group of inherited, autosomal recessive disorders
 Presence of an abnormal form of hemoglobin in the
erythrocyte
 Hemoglobin S (HbS), abnormal
Sickle Cell Disease (Cont’d)
 HbS causes the RBC to stiffen and elongate
 Sickle shape in response to ↓ O2 levels
 Substitution of valine for glutamic acid on the β-globin chain
of hemoglobin
 Genetic disorder
 Incurable disease, often fatal
Sickle Cell Disease
Etiology and Pathophysiology
 Types of SCD
 Sickle cell anemia
Most severe
 Homozygous for hemoglobin S (HbSS)




Sickle cell thalassemia
Sickle cell HbC disease
Sickle cell trait (HbAS)
Sickle Cell Hemoglobin Aggregates and Alters
Shape of RBC
Sickle Cell Disease
Clinical Manifestations
 Typical patient is asymptomatic except during
sickling episodes
 Symptoms can be



Pain and swelling
Pallor of mucous membranes
Fatigue
Clinical Manifestations of Sickle Cell Disease
Sickle Cell Disease
Complications
 Gradual involvement of all body systems
 Usually fatal by middle age from renal and
pulmonary failure
 Prone to infection

Pneumonia, most common infection
Sickle Cell Disease
Diagnostic Studies
 Peripheral blood smear
 Sickling test
 Electrophoresis of hemoglobin
 DNA testing
 Skeletal x-rays
 Magnetic resonance imaging (MRI)
Sickle Cell Disease
Nursing Management
 Alleviate symptoms of disease complications
 Minimize end-organ damage
 No specific treatment for SCD
 Patient teaching
 Avoid high altitudes, maintain fluid intake, treat infections,
control pain
Sickle Cell Disease
Nursing Management (Cont’d)
 O2 for hypoxia and to control sickling
 Pain management
 Acute chest syndrome
 Antibiotics
 O2 therapy
 Fluid therapy
Sickle Cell Disease
Nursing Management (Cont’d)
 Folic acid daily supplements
 Blood transfusions in crisis
 Hydroxyurea: Antisickling agent
 Erythropoietin in patients unresponsive to hydroxyurea
 Bone marrow transplant
 Can cure some patients with SCD
Care plan for anemia
 Example of Nursing Diagnosis

Fatigue r/t decreased oxygen supply to the body, increased cardiac
workload
 Example of Outcome

Identify potential factors that aggravate and relieve fatigue by the
end of the shift as evidenced by patient self identifying activities that
cause increased fatigue
 Intervention


Assess severity of fatigue level on scale of 0-10
Assess frequency, activities, and symptoms associated with increased
fatigue
Ackley
Care plan for anemia
 ND: Impaired gas exchange r/t lack of RBC’s,
hemoglobin abnormalities
 Outcome: within 3-24 hours patient has adequate
gas exchange as evidenced by HR and RR within 10%
of baseline
 Intervention: Supplemental oxygen, monitor HR and
RR, Monitor oxygen saturation
Baird, Ch 10
Hemolytic Anemia
 Destruction or hemolysis of RBCs at a rate that
exceeds production
 Third major cause of anemia
 Intrinsic hemolytic anemia



Abnormal hemoglobin
Enzyme deficiencies
RBC membrane abnormalities
Transfusion Responsibilities
 Pretransfusion:
 Verify prescription
 Test donor’s/recipient’s blood for compatibility
 Examine blood bag for identification
 Check expiration date
 Inspect blood for discoloration, gas bubbles, cloudiness
Transfusion Responsibilities (cont’d)
 Provide patient education
 Assess vital signs
 Begin transfusion slowly, stay with patient first
15 to 30 minutes
 Ask patient to report unusual sensations (e.g.,
chills, shortness of breath, hives, itching)
 Administer blood product per protocol
 Assess for hyperkalemia
Transfusion Reactions
 Febrile
 Anti WBC-antibodies
 Multiple transfusions of PRBC, WBC, and/or platelet
transfusions
 Develops chills tachycardia fever hypotension and tachypnea
 Prevention WBC filters premedication's
 Review local policies r/t fever definition
Transfusion Reactions
 Hemolytic
 Blood type or Rh incompatability





Antibody-antigen reaction
Mild to severe symptoms up to Disseminated intravascular
coagulation (DIC)
May be immediate or delayed
Manifested by apprehension, H/A, chest pain, low back pain,
tachycardia, hypotension, hemoglobinuria,
STOP THE TRANSFSION IMMEDIATELY !!!!!

Replace the entire IV infusion system from patient to bag, follow
local policy r/t post reaction testing
Transfusion Reactions
 Allergic (anaphylactic)
 May see urticaria, itching bronchospasm or anaphylaxis
 Give leukocyte poor WBC, filtered, and/or irradiated blood to
patients with history
 If anaphylaxis occurs this is an emergent situation, call a
code, stop the transfusion and follow code procedures
dependent on situation.
Transfusion Reactions
 Bacterial
 Contaminated blood products
 Can lead to sepsis with symptoms of tachycardia, hypotension,
fever, chills and shock
 Prevention by keeping blood products chilled
Multiple Myeloma
 Pathophysiology
 Overproduction of the B-lymphocyte that produce antibodies
and cytokines leading to bone destruction, clogging of blood
vessels in kidney and other organs and decreased amounts of
other cells produced in the marrow
 May be asymptomatic but may progress to feelings of fatigue,
easy bruising and bone pain. May have unexplained fractures,
HTN, and infections
 Testing will show elevated serum proteins and
immunoglobulin's, X-ray will show thinning bones
Multiple Myeloma
 Interventions
 Dependent on severity
 Chemotherapy, steroids, bone marrow transplant
 Supportive care is targeted to teaching about the
toxic effects of medications and for those
hospitalized, based on cell counts, isolation to
protect from infections
Purpose of Inflammation &
Immunity
Meet human need for protection by neutralizing,
eliminating, or destroying organisms invading the
internal environment
Organization of the Immune System
 Immune system influenced by many systems
(e.g., nervous system, endocrine system, GI
system)
 Stem cells – Immature, undifferentiated cells;
produced by bone marrow
 Leukocytes (WBCs) – Protect body from effects
of invasion by organisms
Stem Cell Differentiation and
Maturation
Inflammation: “Innate Native
Immunity”
 Any natural protective feature of a person
 Provides immediate protection against effects of
tissue injury and foreign proteins—critical to
health and well-being
 Causes visible symptoms and can rid body of
harmful organisms; tissue damage may
result from excessive response
Cell Types Involved in Inflammation




Neutrophils
Macrophages
Basophils
Eosinophils
Sequence of Inflammatory Responses
 Five cardinal manifestations of inflammation:
 Warmth
 Redness
 Swelling
 Pain
 Decreased function
Sequence of Inflammatory Responses
(cont’d)
 Stage I (vascular) – Change in blood vessels:
 Phase I—constriction
 Phase II—hyperemia & edema
 Stage II (cellular exudate) – Neutrophilia, pus
 Stage III (tissue repair and replacement) –
WBCs trigger new blood vessel and growth
(angiogenesis) and scar tissue formation
Rheumatoid Arthritis (RA)
 Common connective tissue disease, destructive
to joints
 Chronic, progressive, systemic inflammatory
autoimmune disease; affects primarily synovial
joints
 Transformed autoantibodies (rheumatoid
factors) form, attack healthy tissue causing
inflammation
RA Pathology
RA Collaborative Management
 Assessment
 Physical assessment/clinical manifestations:
 Early—joint stiffness, swelling, pain, fatigue, generalized
weakness
 Late—joints become progressively inflamed and quite
painful
RA Joint Involvement
RA Systemic Complications







Weight loss, fever, extreme fatigue
Exacerbations
Subcutaneous nodules
Respiratory, cardiac complications
Vasculitis
Periungual lesions
Paresthesias
RA Assessments
 Psychosocial
 Laboratory—rheumatoid factor, antinuclear
antibody titer, ESR, serum complement (C3 &
C4), serum protein electrophoresis, serum
immunoglobulins
 Thrombocytosis can occur with late RA
 Other diagnostic—x-ray, CT, arthrocentesis,
bone scan
RA Drug Therapy
 Disease-modifying antirheumatic drugs
(DMARD)

Methotrexate (MTX)
 NSAIDs
 Biological response modifiers (BRM)
 Neutralize alpha tumor necrosis factor (Humira,
Enbrrel)
 Other:
 Glucocorticoids
 Immunosuppressive agents
(See pages 338-339 of Iggie)
RA Nonpharmacologic Interventions








Adequate rest
Proper positioning
Ice and heat application
Plasmapheresis (not common)
Complementary and alternative therapies
Promotion of self-management
Management of fatigue
Enhance body image
Care of RA
 Promotion of self management
 Manage fatigue
 Enhance body image
 Self-management
 Home care
Lupus Erythematosus
 Chronic, progressive, inflammatory connective





tissue disorder
Can cause major body organs/systems to fail
Spontaneous remissions and exacerbations
Autoimmune process
Autoimmune complexes tend to be attracted to
glomeruli of the kidneys
Often some degree of kidney involvement
Lupus Clinical Manifestations
 Skin involvement
 Butterfly rash
 Polyarthritis
 Osteonecrosis
 Muscle atrophy
 Fever and fatigue
Lupus Clinical Manifestations (cont’d)






Renal involvement
Pleural effusions
Pericarditis
Raynaud’s phenomenon
Neurologic manifestation
Serositis
Characteristic “Butterfly” Rash of
SLE
Assessments for Lupus
 Psychosocial results can be devastating
 Laboratory:
 Skin biopsy (confirms diagnosis)
 Immunologic-based laboratory tests
 CBC (often shows pancytopenia)
 Body system function
SLE Drug Therapy






Topical cortisone drugs
Plaquenil
Tylenol or NSAIDs
Chronic steroid therapy
Immunosuppressive agents
New drugs in clinical trials


Lupozor
Belimumab (Benlysta)

Monoclonal antibody medication
Care of SLE patients
 Similar to RA
 Also teach skin protection
 monitoring of body temperature
 increased temperature could be sign of exacerbation
 Family education
Youtube videos of interest (cut-n-paste)
Anemia
Multiple myeloma
http://www.youtube.com/watch?v=pGTu
2aDbLpg
http://www.youtube.com/watch?v=2VJIg
RO7Yag
http://www.youtube.com/watch?v=SP7M
CBGyYfQ
http://www.youtube.com/watch?v=Jycz1
WBs-QQ
Lupus
http://www.youtube.com/watch?v=aGgV
CRg3OHI
http://www.youtube.com/watch?v=6_Uyf
3op8DI
Autoimmunity
http://www.youtube.com/watch?v=0z1qqf
4Ekb0
Rheumatoid arthritis
http://www.youtube.com/watch?v=0uwx
64YaxSk
http://www.youtube.com/watch?v=EPmF
4vWkuCk
http://www.youtube.com/watch?v=mIewj
ILmG5M
Transfusion reactions
http://www.youtube.com/watch?v=frYwX
cLv5yc