1.

Red Blood Cell Disorders

1.1. Anemias
1.2. Myelodysplastic Syndrome
1.3. Polycythemia Vera
1.1. Iron Deficiency Anemia
1.2. Pernicious Anemia
1.3. Aplastic Anemia
1.4. Hemolytic Anemia
1.5. Sickle Cell Anemia
1.6. Splenectomy

1. Anemias
 1. Anemia’s
 Reduction of  Types in Children
◦ Number of red ◦ Iron-deficiency
blood cells Anemia
(erythrocytes) ◦ Sickle Cell Anemia
◦ The quality of ◦ Beta-Thalasemia
hemoglobin Major (Cooley’s
◦ The volume of Anemia)
packed red
 It is a condition that is slowly rising in
cases across all countries.
 Every age and every stage can be
affected by this blood condition.

 It is a condition in which the hemoglobin

concentration is lower than normal.

 It is NOT a specific disease state but can

be an underlying disorder and the most
common hematologic condition

Introduction: Anemia
 Anemia is usually define as a
decrease in the amount od
red blood cells or the amount
of hemoglobin in the blood.

 It
also defined as a lowered
ability of the blood to carry
oxygen.

Introduction
 Iron deficiency anemia

 Pernicious anemia

 Folic acid deficiency anemia

 Aplastic anemia

Most Common Anemias in Adult

 Blood loss
 Decreased or faulty red
blood cell production
 Destruction of RBCs
 Fluid overload

What Causes Anemia?

 Fatigue
 Weakness
 Pale skin
 Fast or irregular heartbeat
 SOB
 Chest pain
 Dizziness
 Cognitive problems
 Cold hands and feet
 HA

Signs and Symptoms

 Central Nervous System
◦ Fatigue
◦ Dizziness
◦ Fainting

 Eyes
◦ Yellowing

 Skin
◦ Paleness
◦ Coldness
◦ Yellowing

Symptoms (Severe Anemia)

 Blood vessels
◦ Low blood pressure

 Heart
◦ Palpitations
◦ Rapid heart rate
◦ Chest pain
◦ Angina
◦ Heart Attack

Symptoms (severe anemia)

 Respiratory
◦ Shortness of breath (SOB)

 Muscular
◦ Weakness

 Intestinal
◦ Changed stool color

 Spleen
◦ Enlargement (Splenomegaly)

Symptoms
 Dietlacking in certain vitamins
(low intake of iron diet. Vitamin
B12 and folate)

 Intestinal disorders
◦ Crohn’s disease
◦ Celiac disease
◦ Removal of the parts of the small
intestine
Risk Factors
 Menstruation (female patient)
◦ This causes the loss of red blood cells

 Pregnancy
◦ Your iron stores have to serve your
increased blood volume as well as be
a source of hemoglobin for growing
fetus/baby.

Risk Factors
 Chronic condition
◦ Ca, kidney or liver failure – there are shortage of
RBC

 Family History
◦ Sickle Cell Anemia

 Other Factors
◦ Hx of certain infections, blood disease, and
autoimmune disorders
◦ Alcoholism
◦ Exposure to toxic chemicals
◦ Use of some medications that affect RBC
production

Risk Factors
Common Types of Anemia
1.1. Iron-Deficiency Anemia
 It is a condition in which the total body
iron content is decreased below the
normal level affecting hemoglobin
synthesis
 This type of anemia caused by:
◦ Blood loss – heavy menstrual bleeding,
ulcer, Ca, polyps in digestive system, &
prolonged use of aspirin, NSAID’s,
◦ Pregnancy
◦ Lack of iron supply in the diet.

Iron-Deficiency Anemia
 The MOST common hematologic disorder
of infancy and childhood
 9 months – 2 years, Adolescence
 A nutrient deficiency of inadequate dietary
iron

 Prevention:
◦ Iron fortified products

Iron-Deficiency Anemia
 Low birth weight infants
 Infants born to mother with
Iron Deficiency Anemia
 Infants born with GI defects
 Chronic blood loss in older
children

Children at Risk
 Iron deficiency anemia is caused by a
shortage of the element iron in the body
 Bone marrow needs iron to make
hemoglobin
 Without adequate iron, our body can’t
produce enough hemoglobin for red blood
cells
 Decreased hemoglobin may result in
insufficient oxygen delivery to the tissue
causing anemia.

Pathophysiology
 HA, dizziness, fatigue, tinnitus

 Palpitations,dyspnea on
exertion, pallor of skin and
mucous membrane

 Indeveloping countries: smooth,

sore tongue, cheilosis, koilonchia
(spoon shape nails) and pica
Clinical Manifestations
 Clinical Manifestations
 Low Hgb
◦ Low oxygen tissue perfusion
 Hgb of 10.2 or less
◦ May seen asymptomatic
◦ Pallor/Pale mucous membrane
◦ Poor muscle tone, decreased activity
◦ Increased HR & RR
 Hgb less than 9.0
◦ Above manifestations plus irritability, lack of
interest in play
 Low RBC’s
 Low HgB
◦ 10.2 (mild), 8-9 (moderate), less than 7
(severe)
 Low Hct
 Low Iron
 High transferrin
 Low ferritin

Laboratory Tests
 Imbalance nutrition: less than body
requirements related to inadequate
intake of iron.

 Activityintolerance related to
decreased oxygen-carrying capacity
of the blood.

 Ineffective
tissue perfusion related to
decreased oxygen-carrying capacity
of the blood.

Nursing Diagnosis
Nursing Interventions
 Promoting iron intake (see next
slide)
 Assess diet for inclusion of foods
rich in iron.
 Arrange nutritionist referral as
appropriate
 Administer iron replacement as
ordered. (Z-track technique)
IRON-RICH FOOD VITAMIN C RICH FOOD

 Meat, fish poultry  Orange juice

 Vegetables  Citrus fruits
 Dried fruits  Strawberries
 Legumes  Tomatoes
 Enriched grain  Broccoli
products  Leafy green
 Whole grain cereal vegetables
 Iron-fortified cereal  Potatoes

Nursing Consideration
Nursing Interventions:
Collaborative Approach
 Earlydiagnosis and correction of
chronic blood loss. (determination
of source of chronic blood loss)

◦ Sigmoidoscopy
◦ Colonoscopy
◦ Upper and lower GI studies
◦ Stool an urine for occult blood
examination
Other Management
 Iron-fortified formula

 Limit cow’s milk to 24-32 oz/day for

children above 12 months

 Increased age-appropriate iron-rich foods

and Vitamin C (see previous slide)

 Fe supplements – Ferrous sulfate

 Manage side effects of FeSO4.
◦ Nausea, Anorexia, Constipation,
Abdominal distress and Black stool

 Give
FeSo4 on an empty
stomach if possible.

 Monitorbowel movements and

increased fluid and fiber intake.
 Monitor development, sleep and activity
or fatigue patterns.

 Monitor Hgb to measure effectiveness of

therapy

 Instruct families/SO to keep FeSO locked

and out of reach of children – POISONING
is a serious risk.

Nursing Management
1.2. Megalobalstic Anemia

1.2.1 Pernicious Anemia

1.2.3. Folic Acid Deficiency

1.2.1 Pernicious Anemia
A megaloblast is a large,
nucleated erythrocyte with
delayed and abnormal
nuclear maturation.

Pernicious Anemia
 PERNICIOUS ANEMIA is a
type of megaloblastic anemia
associated with vitamin B12
deficiency because of lack of
intrinsic factor in gastric
secretion.

Pernicious Anemia
 VitaminB12 is necessary for
normal deoxyribonucleic acid
synthesis in maturing RBCs.

 Perniciousanemia demonstrate
familial incidence related to
autoimmune gastric mucosal
atrophy.
Etiology or Cause
 Normal gastric mucosa secretes a
substance called INTRINSIC
FACTOR”, necessary for absorption
of vitamin B12 in ileum. It a defect
exist in gastric mucosa, or after
gastrectomy or small bowel
disease, this intrinsic factor may
not ne secreted and orally ingested
B12 not absorb.
Etiology or Cause
 Some drugs interfere Vitamin
B12 absorption, notably ascorbic
acid, Cholestyramine, colchicine,
neomycin, cimetidine, and
hormonal contraceptives.

 Primarily
a disorder of “OLDER
PEOPLE”.

Etiology or Cause
 PerniciousAnemia, disease in
which the production of red
blood cells (erythrocytes) is
impaired as the result of the
body’s inability to absorb vitamin
B12 which is necessary for the
RBC to mature properly in the
bone marrow.

Pathophysiology
 Perniciousanemia is one of
many types of anemia, a disease
marked by a reduction in RBC or
in the oxygen-carrying
substance hemoglobin found in
those cells.

Pathophysiology
 Pallor,
fatigue, dyspnea on
exertion, palpitations. Maybe
angina pectoris and heart failure
in the elderly or those
predisposed to heart disease.

Clinical Manifestations
 Pallor
 Fatigue
 Dyspnea on exertion,
 Palpitations

◦ Maybe angina pectoris and heart

failure in the elderly or those
predisposed to heart disease.

Clinical Manifestations
 GI dysfunction:
◦ Sore mouth
◦ Glossitis
◦ Anorexia
◦ Nausea
◦ Vomiting
◦ Loss of weight
◦ Indigestion
◦ Epigastric discomfort
◦ Recurring diarrhea or constipation

Clinical Manifestations
 Neuropathy:
◦Paresthesia that involves hands
and feet.
◦Gait disturbance
◦Bladder and bowel dysfunction
◦Psychiatric symptoms caused by
cerebral dysfunction

Occurs in high percentage of

untreated patients.

Clinical Manifestations
 Complete blood count (CBC)
◦ Hemoglobin – protein bound to
oxygen to carry it throughout the
body

◦ Hematocrit – used to measure how

much space RBC use within the blood.

 Vitamin B12 deficiency test

 Intrinsic factors, absent

Diagnostic Evaluation
 Disturbed thought process
related to neurologic dysfunction
in absence of vitamin B12.

 Impairedsensory perception
(kinesthetic) related to
neurologic dysfunction in
absence of vitamin B12.
Nursing Diagnosis
 Improving thought processes
◦ Administer parenteral vitamin B12
as Rx
 Hydroxocobalamin
 Cyanocobalamin (B12)

◦ I.M. injection
◦ Generally given every month

Nursing Interventions
 Improving thought processes
◦ Provide patient with quiet,
supportive environment.
◦ Reorient to time, place and person
if needed.
◦ Give instruction and information in
short, simple sentences and
reinforce frequently.

Nursing Interventions
 Minimizingthe effects of
paresthesia
◦ Assess extent and severity of
paresthesia, imbalance, or other
sensory alterations.

◦ Refer patient to physical and

occupational therapy as appropriate.

◦ Provide safe, uncluttered environment

Nursing Interventions
 Minimizingthe effects of
paresthesia
◦ Make sure personal belongings are
within reach.

◦ Provide assistance with activities as

needed.

Nursing Interventions
 Advisepatient that monthly vitamin
B12 administration should be
continued for life.

 Instruct
patient to see health care
provider approximately every 6
months for hematologic studies &
GI evaluation; may develop
hematologic or neurologic relapse if
therapy inadequate.
Patient Education and
Maintenance
1.2.3. Folic Acid Deficiency
 Chronic megaloblastic anemia
caused by folic acid (folate, B9)
deficiency.

Folic Acid Deficiency

 Dietary
deficiency, malnutrition,
marginal diets, excessive
cooking of foods

 Alcoholism

 Impairedabsorption of iron in
jejunum (e.g. with small bowel
disease)
Etiology
 Increasedrequirement (eg.
Pregnancy, exfoliative dermatitis,
chronic hemolytic anemia).

 Impairedutilization from folic acid

antagonists (Methotrexate).

 Otherdrugs: (phenytoin, broad

spectrum antibiotics,
sulfamethoxazole, alcohol,
hormonal contraceptives
Etiology
 Of Anemia
◦ Fatigue
◦ Weakness
◦ Pallor
◦ Dizziness
◦ Headache
◦ tachycardia

Clinical Manifestations
 Of Folic Acid Deficiency
◦ Sore tongue
◦ Cracked lips

Clinical Manifestations
 VitaminB12 and Folic Acid level
– decreased

 Decreased RBC, Hemoglobin,

hematocrit

 Increased
Mean Corpuscular
volume and mean corpuscular
hemoglobin concentration
Diagnostic Evaluation
 Imbalance nutrition: less than
body requirements related to
inadequate intake of folic acid

Nursing Diagnosis
 Improving Folic Acid Intake

◦ Assess diet for inclusion of food rich in folic

acid: such as beef liver, peanut butter, red
beans, oatmeal, broccoli & asparagus.

◦ Assist alcoholic patient to obtain counseling

and additional medical care as needed.

◦ Arrange nutritionist referral as appropriate.

◦ Give oral folic acid replacement on daily basis

 Folic acid (Folvit)

Nursing Interventions
 Encourage pregnant patient to
maintain prenatal care and to take
folic acid supplements.

 Providealcoholic patients with

information about treatment
programs and Alcoholic Anonymous
meetings in the community.

 Patient
education and health
maintenance.
Community & Home Care
Considerations
 Teach patient to select balance diet
that includes green leafy vegetables
(asparagus, broccoli, spinach),
yeast, liver and other organ meats,
some fresh fruits.

 Avoid overcooking of vegetables.

 Encourage patient to follow up

periodically to monitor CBC.
Community & Home Care
Considerations
1.3. Aplastic Anemia
 Thisvery rare life-threatening
anemia is caused by bone
marrow hypoplasia or aplasia
resulting in pancytopenia
(insufficient numbers of RBCs,
WBCs and platelets

Aplastic Anemia
 Chemical toxins such as
pesticides, arsenic, and benzene.

 Radiation
and chemotherapy
(treatment for cancer)

 Drugs such as chloraphenicol

(rarely used antibiotics in the
USA)
Causes:
 Infectious
diseases: hepatitis,
Epstein-Barr virus,
cytomegalovirus, parvovirus B19 &
HIV

 Autoimmune disorder: lupus and

RA

 Pregnancy – often goes away after

delivery.

Causes:
 Body malaise, pallor and associated
symptoms such as palpitation.

 Low platelet count (thrombocytopenia)

leading to increased risk of hemorrhage,
bruishing and petechiae.

 Low WBC count (leukopenia) leading tot

increased risk of infection.

 Low counts of reticulocytes

(reticulocytopenia – immature RBC)

Clinical Manifestations
 CBC and Peripheral Blood Smear
◦ Decreased RBC, WBC & platelets

 Bonemarrow aspiration and

biopsy
◦ Hypocellular or empty with greatly
reduced or absent hematopoiesis.

Diagnostic Evaluation
 Risk
for infection related to
granulocytopenia secondary to
bone marrow aplasia

 Riskfor injury related to severe

bleeding

Nursing Diagnosis
 Remove of causative agent or toxin.

 Allogenicbone marrow
transplantation (BMT) – treatment of
choice for severe aplastic anemia
(survival rate 75%-90% depending on
the patient age, hx of prior BT and
source of marrow.

 Immunosuppressive treatment
◦ Cyclophosphamide or Antirhymocyte
globulin (survival rate 60%-70%)

Management
 Androgens (oxymetholone or
testosterone enanthate) may
stimulate bone marrow
regeneration, observed toxicity.

 Supportive treatment includes

platelets and RBC transfusion,
antibiotics and antifungals
Management
 Minimizing risk of infection
◦ Placed on the private room with strict
hand washing and avoidance of any
contaminants.
◦ Encourage good personal hygiene: daily
bath with mild soap, mouth care &
perirectal care.
◦ Monitor VS, esp. body temperature.
◦ Minimize invasive procedure or possible
trauma to skin or mucous membrane.
◦ Obtain cultures of suspected sites or body
fluids.
Nursing Interventions
 Minimizing risk of bleeding
◦ Use only soft toothbrush for mouth care and
electric razor for shaving, keep nails short by
filing.
◦ Avoid IM injections
◦ Prevent constipation with stool softener as
Rx.
◦ Restrict activity based on platelet count and
active bleeding
◦ Monitor pad count for menstruating patient,
avoid use of vaginal tampons
◦ Control bleeding by applying pressure to site,
using icepacks and RX topical hemostatic
agents.
◦ Administer blood products.
Nursing Interventions
 1.4. Hemolytic Anemia

1.4.1. Sickle Cell Anemia

1.4.2. Thalassemia anemia
1.4.1. Sickle Cell Anemia
Itis inherited form of
anemia – a condition in
which there aren’t
enough healthy RBCs to
carry adequate oxygen
throughout the body.

Introduction: Sickle Cell Anemia

 Normally,the RBC are flexible
and round, moving easily
through the blood vessels.

 Insickle cell anemia, the

RBCs become rigid and sticky
and are shaped like sickles or
crescent moon.

Introduction: Sickle Cell Anemia

 These irregularity shaped cells can
get stuck in small blood vessels,
which can slow or block blood flow
and oxygen to parts of the body.

 There is NO cure for most people

with sickle cell anemia. However,
treatments can relieve pain and help
prevent further problems associated
with Sickle Cell Anemia.

Introduction: Sickle Cell Anemia

 Autosomal recessive disorder, African
Americans

 Characterized by abnormal hemoglobin

 Clinical manifestations caused by

obstruction due to sickled RBC’s and
destruction of sickled and normal
RBC’s.

Sickle Cell Anemia (SCA)

 Genetic factor
◦ If both parent has sickle cell trait

 1 child - 100%
 1 child – 50/50%
 1 child – Does not have sickle cell
trait and has normal gene

Causes
 The loss of RBC elasticity in central
to the disease process of sickle-cell
disease. Normal RBC are quite
elastic, which allows the cell to
deform to pass through capillaries.
In sickle-cell disease, low oxygen
tension promotes RBC sickling and
repeated episodes of sickling
damage the cell membrane and
decrease the cell’s elasticity.
Pathophysiology
 These cells fails to return to
normal shape when normal
oxygen tension is restored. As a
consequence, these rigid blood
cells are unable to deform as
they pass through narrow
capillaries, leading to vessel
occlusion and ischemia.

Pathophysiology
 The actual anemia of the illness is
caused by hemolysis, the
destruction of the red cells,
because of their shape. Although
the bone marrow attempts to
compensate by creating new red
cells, it does not match the rate of
destruction. Healthy RBCs typically
function for 90-120 days, but
sickled cells only last 10-20
days.

Pathophysiology
 Severe pain
◦ Chest pain
◦ Joint pain
 Difficulty of breathing
 Severe infections
 Arthritis and bone infarctions
 Vision problems
 Delayed growth

Signs and Symptoms

 Low hematocrit level
 Low sickled cells on the smear
 Increased reticulocytes –
common in children

Diagnosis is confirmed by
hemoglobin
electrophoresis

Diagnostic Evaluation
 Peripheral
Blood Stem Cell
Transplant (PBSCT)
◦ It may cure sickle cell anemia.
However, this treatment modality is
available to only a small subset of
affected patients, because of either
the lack of a compatible donor or
because of severe organ damage
(renal, liver, lung) that may be
already present in the patient is
contraindicated for PBSCT.
Medical Management
 Pharmacologic Therapy
◦ Hydroxyurea (Hydrea) – a
chemotherapy agent has been
shown to be effective in increasing
fetal hemoglobin levels in patient
with Sickle Cell Anemia, thereby
decreasing the formation of sickle
cells.

Medical Management
 Transfusion Therapy
◦ RBC transfusion therapy has been
shown to be highly effective in
several situations:
 In acute exacerbation of anemia
 In the prevention of severe
complications from anesthesia and
surgery
 In improving the response to
infection

Medical Management
 Supportive Therapy
◦ Supportive care is equally important.
Pain management is a significant
issue. The use of medication to
relieve is important.

◦ Aspirin is very useful in diminishing

mild to moderate pain and also
diminishes inflammation and potential
thrombosis
◦ NSAIDs are useful for moderate pain
or combination with opioid analgesics

Medical Management
 Acute pain related to tissue
hypoxia associated with
agglutination of sickled cell with
in blood vessels.
 Administer oxygen via non-
rebreather mask

 Risk for infection

Nursing Diagnosis
 Managing Pain

◦ Guide the rate of pain using pain

scale.
◦ Apply relaxation techniques,
breathing exercise and distraction
to the patient
◦ Provide analgesic agent as Rx.

Nursing Interventions
 Prevention and Managing
Infection

◦ Monitor patient for signs and

symptoms of infection.
◦ Assess for the signs of dehydration
◦ Provide the Rx antibiotic.

Nursing Interventions
 Promoting Coping Skills

◦ Establish therapeutic relationship to

the patient.
◦ Encourage patient on their strength
rather than deficit.
◦ Allow patient to make decisions
about daily care and explain the
rationale.
Nursing Interventions
 Minimizing deficient knowledge

◦ Explain all the potential side effects

of the used drugs.
◦ Give the sufficient knowledge about
the disease process to the patient.

Nursing Interventions
 Promoting home and Community
based care

◦ Provide knowledge on self care and

hygiene.
◦ Provide follow up care.
◦ Encourage for communication.
◦ Guidelines should be given regarding
when to seek the urgent care.

Nursing Interventions
 Vaso-Occlusive Crisis

 Acute Chest Syndrome

 Splenic Sequestration

3 Sequalea of SCA
 Severe, sudden onset of sickling
where many new sickled cells pool in
a vessels and cause pain and
tissue hypoxia.
 Caused by: infection, dehydration,
anxiety and cold
 Most common from hypoxia
secondary to rapidly destroyed RBC
 Lasts for hours to weeks

A. Vaso-Occlusive Crisis
 Early Signs
◦ Pallor, tachycardia and fever
 Late Signs
◦ Acute abdominal, back and extremity
pain
 First Crisis in Infants
◦ Dactylitis (hand & foot syndrome)
 Swelling of hands and feet
 Joints maybe warm and swollen

Clinical Manifestations
 Pain relief

 Adequate hydration

 Adequate oxygenation

Management
 Assess pain every 1-2h or more
frequently
 Use pain scale appropriate for age
 Non-pharmacological pain methods
 Around the clock pain medications
◦ Tylenol for mild pain

◦ Narcotics for moderate-severe pain

Pain
 Push by mouth (p.o.) fluids

 IVhydration: 1.5 – 2 times normal

rate

 Risk for fluid overload

Prevent Occlusion
Prevent Further Sickling &
Altered Tissue Perfusion
 Administer oxygen to maintain
saturation of 95% or higher

 Pulse oximetry

 Semi-fowler’s position

 Administer Packed RBC’s

 Sickle contents break off

 Bilateral pulmonary involvement

 Causeschest infection and

embolism

B. Acute Chest Syndrome

 Chest pain
 Fever
 Cough
 Wheeze
 Tachypnea

Clinical Manifestations
 Hydration
 Oxygen
 Incentive spirometry
 Analgesics
 Antibiotics
 PRBC

Management
 Sickled cells block the spleen
 Pooled blood in spleen and/or liver
and enlarges
 Pooled blood leads to a decrease in
circulating volume resulting
hypovolemic shock
 CVA leads to COMA

C. Splenic Sequestration
Irritability
Pale
Tachycardia
Pain to LUQ
Enlarged spleen

Clinical Manifestations
 Life
Threatening
◦Get child to ED a.s.a.p

 Administer PRBC

 Remove spleen
(spleenctomy)

Management
1.4.2. Thalassemia Anemia
Thalassemia Anemia
 Hereditary anemia due to abnormal
synthesis of hemoglobin

 Lifelong disorder

 Mediterranean descent

 Life threatening symptoms

 Low RBC’s

 Extremely low Hgb – less than 5.0

 Increased serum Iron (Fe)

Diagnostic tests
 Facial anomalies
◦ Frontal bossing (prominent and protruding
forehead)
◦ Maxillary prominence
◦ Wide-set eyes with a flattened nose
 Bronze skin color (greenish yellow skin
tone)
 Growth and maturation retardation

Clinical Manifestations
 RBC transfusion every 2-4 weeks to get
Hgb to 10-12
 Iron chelation therapy
◦ Desferal (deferoxamine) SQ
 Splenectomy

 CURE: bone marrow stem cell transplant

◦ Estimated 70% do not find a suitable donor

Management
 Observe for complications of transfusion-
iron overload
 Supporting the child and family in dealing
with a chronic life-threatening illness
 Monitor growth and development
 Refer the family for genetic counseling

Nursing Considerations
1.2. Myelodysplastic Syndrome
 Heterogenous group of clonal hematopoietic
stem cell disorders.

 In MDS, the bone marrow cannot produce

blood cells effectively, and many of the
blood cells formed are defective.

 MDS is best considered a preleukemic

disorder in which the neoplastic clone that
has been established may or may not fully
progress to acute leukemia.

Myelodysplastic Syndrome
 Varyingdegree of tri-lineage
cytopenia

 Dysplasia

 Normocellular or Hypercellular

 May progress to acute leukemia

Characteristics of MDS
1. Disease of elderly
2. Median age 65 years
3. <10% are younger than 50 years
4. Incidence rates 1/100,000
population/years
5. Male slightly higher than female

Incidence
 Hereditary

 Acquired
◦ Mutagen exposure
◦ Environmental or occupational
exposures
◦ Tobacco

Predisposing Factors
 Excessive tiredness, SOB, pale skin –
caused by anemia.

 Serious infection with high grade fever

caused by leukopenia.

 Excessive bruising and bleeding: nose

bleeds, bleeding gums due to
thrombocytopenia

Signs and Symptoms

 Splenomegaly & hepatomegaly

 Cutaneous
manifestations:
Uncommon
◦ Sweet’s syndrome (neutrophic dermatosis)

◦ Granulocytic sarcoma (Chloroma): a disease

transformation into acute leukemia

Signs and Symptoms

 Complete blood count (CBC)

 Bone marrow aspiration & biopsy

 Bone marrow cytogenetics analysis

 Granulocyte & Platelet Function test

 Serum protein electrophoresis

Diagnostic Evaluation
 Supportive therapy (transfusion)

 Hypomethylating agents
◦ Azacitidine
◦ Decitabine

 Immunosuppression
◦ Antithymocyte globulin
◦ Cyclosporin
◦ Thalidomide

 Chemotherapy

 Allogeneic stem cell transplantation

Management
1.3. Polycythemia Vera
 It
is a blood disorder in which the
body produces too many blood cells
as a result of a problem with the bone
marrow or an increased production of
the hormone erythropoietin (EPO).

 It
is also defined as an increase in the
hemoglobin above normal.

Polycythemia: Definition
 An increase in the number of RBC is
called ABSOLUTE POLYCYTHEMIA.
1. Primary
2. Secondary

2.1. Appropriate
2.1. Inappropriate

 Due to decrease in the volume of plasma

is called RELATIVE POLYCYTHEMIA.

Polycythemia: Classification
 Over production of RBC may be due
to a primary process in the bone
marrow called myeloproliferative
syndrome.

 Maybe a reaction to chronically low

oxygen levels

 Over transfusion

Absolute Polycythemia
 It is an apparent rise of the erythrocyte
level in the blood.

 The underlying cause is reduced blood

plasma.

 It is often cause by loss of body fluids or

low volume states such as through burns,
dehydration, prolonged vomiting, diarrhea,
excessive diuretics and stress or Gaisbock
Syndrome.

Relative Polycythemia
 It is a slow-growing type of blood
cancer in which the bone marrow
makes too many RBC.

 It may also results in production of too

many of the other types of blood cells
– WBC and platelets

 Also called Vaquez’ Disease.

Polycythemia Vera
 It is one of the chronic myeloproliferative
disorders (neoplasm), collectively
characterized by clonal proliferation of
myeloids cells.

 The most prominent feature of this disease

is an elevated absolute RBC mass because
of uncontrolled RBC production

 This is accompanied by increased WBC and

platelet production as well.

Polycythemia Vera
 Age
◦ common in adults and older than 60.
◦ Rare in people younger than 20
 Sex
◦ Affects men more than it does in women
 Family History
◦ Genetic factors other than JAK2 (Janus Kinase
2)

Risk Factors
 Earlystage manifestation:
asymptomatic
 Disease progression:
◦ Headache
◦ Dizziness
◦ Itchiness, especially following a warm bath or
shower – (Pruritus - late symptoms due to
abnornal histamine metabolism)
◦ Skin Redness or Plethoric facial appearance
◦ SOB and DOB when lie down

Signs and Symptoms

 Disease progression: cont.
◦ Numbness, tingling, burning or weakness of
hands, feet, arms and legs
◦ Chest pain
◦ Feeling of fullness or bloating of upper
abdomen due to enlarge spleen
◦ Fatigue

Signs and Symptoms

 Bleeding (epistaxis, gum bleeding, Ecchymosis, & GI
bleeding)

 Thrombotic (thromboembolism or possible Budd-

Chiari Syndrome-hepatic portal vein thrombosis)

 Abdominal pain (PUD due to increase histamine levels

and gastric acidity)

 Splenomegaly (impaired gastric filling)

 Pruritus due to increase histamine levels released

from increase basophils and mast cells.

Signs and Symptoms

 Bone marrow aspiration and biopsy
 Blood Tests:
◦ Increase in the number of RBC, in some cases –
increase in platelets or WBC

◦ Elevated hematocrit measurement

◦ Elevated levels of hemoglobin

◦ Lower than normal levels of oxygen

◦ Very low levels of Erythropoietin (EPO)

Diagnostic Evaluation
 Phlebotomy – first treatment option.
 Medications/Drugs to suppress the bone
marrow’s ability to produce blood cells
◦ Hydroxyurea
◦ Anagrelide
◦ Radioactive phosphorus
 Low dose of aspirin – reduce blood clots
and burning pain
 Therapy to reduce itching
◦ Antihistamine
◦ H-2-receptors
◦ Ultraviolet light to relieve discomfort

Management
 Ruxolitinib
◦ Trade name: jakafi
◦ JAK1/JAK2 inhibitors

Second Line Therapy

 2.
White Blood Cell Disorders

2.1. Neutropenia
2.2. Agranulocytosis
2.1. Neutropenia
 Decreased neutrophil counts

 Types:
◦ Mild Neutropenia
◦ Moderate Neutropenia
◦ Severe Neutropenia

Neutropenia: Definition
 WBC’s fight foreign bodies

 Increased risk for infection

Why is it important to care for

Neutropenic patients?
 Cleanliness
◦ Apply seven steps of hand washing.
◦ Apply 5 moments of hand washing.

 Restrict visitors, “NO flowers”.

 Give neutrophil diet such as banana,

orange. “NO raw food”.

 Check vital signs esp. body temperature,

inform Dr. if fever present

Management
 Afebrile

Low Risk Org. Identified

After 48 hours Cont. tx for 7 days

Oral ciplo + amoxy
+ Clavunate
• Culture –ve
• Infection site resolve
DISCHARGE • Mucosa intact
• NO invasive procedure

Febrile Neutropenia
 Wear PPE.

 Protectpatient from getting

infection.

Reverse Barrier Nursing

2.2. Agranulocytosis
 It
is also known as agranulosis
or granulopenia.

 Anacute condition involving a

severe and dangerous
leukopenia (lowered WBC), most
commonly of neutrophils causing
a neutropenia in the circulating
blood.
Agranulocytosis: Definition
 Itis a severe lack of one major
class of infection-fighting WBC.

 People with this condition are

very high risk of serious
infections due to their
suppressed immune system

Agranulocytosis: Definition
 InAGRANULOCYTOSIS, the
concentration of granulocytes (a
major class of WBC that includes
neutrophils, basophils and
eosinophils) drops below 500
cells/mm3 of blood.

Agranulocytosis: Definition
 Agranulocytosis is an uncommon
condition in which bone marrow
doesn’t make enough
neutrophils.
 Neutrophils are WBC the body
needs to fight infections. They
make up the largest portion of
WBC in the body.

.
 Agranulocytosis can be
congenital, meaning you’re born
with the condition. You can also
acquire it from certain drugs or
medical procedures

Congenital

Acquired

Classification
 Congenital
 Acquired
◦ Chemotherapy or a bone marrow
transplant
◦ Certain medications/drugs:
 antipsychotic
 drug for hyperthyroidism.
◦ Autoimmune disorder:lupus and RA
◦ Bone marrow disease: myelodysplasia
◦ Certain infections: HIV and Hepatisis)

Causes
 Sudden fever, HIGH grade fever
 Chills
 Sore throat
 Weakness in the limbs
 Mouth ulcers
 Bleeding gums

Early Symptoms
 Fast heart rate

 Rapid breathing

 Low blood pressure

 Skin abscesses

Other Signs and Symptoms

 The Cheapest
Investigation!

BLOOD ANALYSIS
(CBC)
Diagnostic Evaluation
 Treat the underlying cause.

 Granulocyte Colony-stimulating factor

◦ Neupogen (filgrastim)
◦ Neulasta (pegfilgrastim)
◦ Granocyte (lenograstim)

 Drugs
◦ Antibiotics

 Immune suppression
◦ Prednisone

 Bone marrow transplant

Management
 Avoid contact with people who have
infections.

 Stay away from crowds.

 Avoidsfruits and vegetables which

cannot be peeled.

 Avoid cut flowers or working in the

soil.

General Measures
 3.
Platelet & Coagulation Disorders

3.1. DIC
3.2.ITP
3.3. Hemophilia
3.4. Von Willebrand’s Disease
3.1. Disseminated Intravascular
Coagulation
(DIC)
 Itis an acquired syndrome
characterized by the intravascular
activation of coagulation with loss
of localization arising from different
causes.

 It
can originate from and cause
damage to the microvasculature,
which if sufficiently severe, can
produce organ dysfunction.
DIC: definition
 Acute DIC
◦ It happened rapidly, the coagulopathy
is dominant and major symptoms are
bleeding and shock, mainly seen in
severe infection, amniotic fluid
embolism.

 Chronic DIC
◦ It happened slowly and last several
weeks, thrombosis and clotting may
predominate, mainly seen in cancer.

DIC: Classification
 Infectious disease: 31% - 43%.
◦ Bacterial, viral, or parasitic
◦ Septicemia – a bacterial infection is
commonly associated with DIC

 Cancer: 24% - 34%

◦ Lung, breast, GI malignancy

 Obstetric Complications: 4% - 12%

◦ Amniotic fluid embolus, septic
abortion

DIC: Causes/Etiology
 Severe tissue injury: 1% - 5%
◦ Burns, heart attack, fracture, head
trauma

 Systemic disease
◦ Malignant hypertension, ARDS,
hemolytic transfusion reaction

DIC: Causes/Etiology
 Stimulation of Coagulation

Intravascular Consumption of Secondary

Coagulation coagulation activation of
factors thrombolysis

Hypoperfusion
to tissues & Inability to form Release of
organs a stable clot anticoagulants

Ischemic
Bleeding Bleeding
damage

Pathophysiology
 Platelet count decreased
 Fibrin degradation product (FDP)
increased
 Factor assay decreased
 Prothrombin time (PT) prolonged
 aPTT prolonged
 Thrombin Time prolonged
 Fibrinogen decreased
 D-dimer increased
 Antithrombin decreased

Diagnostic Evaluation
 Organ Ischemic Hemorrhage
Skin Gangrene, Acral Petechiae,
cyanosis, pruritus echymosis,
oozing
CNS Delirium, coma, Intracranial
dizziness bleeding
Renal Oliguria or Hematuria
Azotemia, cortical
necrosis
GI Ulcers, infarcts Massive

Clinical Manifestations
 Organ Ischemic Hemorrhage
Cardiovas- Myocardial NONE
cular dysfunction

Pulmonary Dyspnea or Hemorrhagic

hypoxia (O2 lung
below 80%)
Endocrine Adrenal infracts Massive

Clinical Manifestations
 Fluid
volume deficit related to
severe bleeding

 Impaired skin integrity

 Coping

Nursing Diagnosis
 Bleeding

 Thrombosis

 Hypotension or shock

 Organ dysfunction

Complications
 Treat the underlying cause.
 Provide supportive management of
complications
 Stop abnormal coagulation and
control bleeding by replacement of
depleted blood and clotting
components
 Medications:
◦ Heparin
◦ Antithrombin III
◦ Fibrinolytic inhibitors

Management
3.2. Idiophatic Thrombocytopenia
Purpura (ITP)
Immune Thrombocytopenic
Purpura
 Autoimmune disorder (antiplatelet
antibody) or cause is UNKNOWN
(idiopathic)
 Occurs most commonly at age 2-4 years
 Reduction in and destruction of platelets
 Typically seen 2 weeks after a febrile, viral
illness
 Excessive bruishing and petechiae
 Epistaxis
 Bleeding into joints
 Tourniquet test:
◦ Shows many petechaie after inflation of BP cuff

Signs and Symptoms

 Laboratory

◦ Paletelets : less than 150 (marked

thrombocytopenia

◦ PT and PTT: Normal

Diagnosis
 Prednisone
 IVIg (IV immunoglobulin)
 Platelet transfusion (only a temporary
solution)
 Most cases are self-limiting
 Avoid when possible:
◦ Administering IM injections
◦ Aspirin, aspirin-containing products &
NSAIDs
◦ Taking temperature rectally
◦ Perform invasive procedures w/ extreme
caution

Management
3.3. Hemophilia A
 Hereditary blood coagulation deficiency
(factor VIII)

 Ability to clot is slower

 X-linked recessive (white, males)

Hemophilia A
 Vary according to
concentration of factor 8
 Soft tissue bleeding
 Painful hemorrhage into
joints
 Severe bleeding may occur in
GI tract, peritoneum or CNS

Clinical Manifestations
 Recent traumas and measures used to
stop bleeding
 Length of time pressure was applied
before bleeding subsided
 Whether swelling increased after surface
bleeding subsided
 Whether swelling and stiffness occurred
without apparent trauma

Subjective Data
 Above history

 Suspected by labs
◦ Platelet level- normal
◦ PTT: prolonged 60 above

 Confirmed by genetic testing for missing

factor

Diagnosis
 Acute Therapy
◦ Bleeding must be controlled by IV
administration of factor 8
 After trauma, surgery
◦ Pressure to laceration
 Prophylactic Therapy
◦ Children age 1-2 receive p.o factor 8
replacement on a regular schedule if frequently
symptomatic
◦ Prior to surgery: dental work

Management of Bleeding
 Primary Goal: Injury prevention
 Promote oral hygiene, up to date
immunizations
 No aspirin
 Avoid activities that induce bleeding
 Provide activities for normal G&D
 Administration of factor replacement prn

Parental Education
3.4. Von Willebrand’s Disease
 Most commonly inherited bleeding
disorder
 Autosomal dominant (Males & Females)
 Lacks production of VWF (chromosome
12)
 Platelets are normal in number
 Inability of platelets to aggregate
 Varying degrees of disease
◦ VWF is deficient to defective

Von Willebrand’s Disease

 Platelets is normal

 PT/PTT is normal

 Conformed by genetic testing for VWF

Diagnosis
 Can be so mild that disease is
UNDIAGNOSED
◦ Epistaxis
◦ Prolonged bleeding from cuts
◦ Excessive bleeding following surgery
◦ Bleeding from gums

Signs and Symptoms

 Prophylactic Therapy
◦ Replace dysfunctional factor in blood
◦ Treatment of choice: DDAVP (desmopressin
acetate) DDAVP = 1-Deamino-8-D-Arginine
VasoPressin) an antidiuretic hormone for
hemophilia A and Von Willebrand Disease
(VWD)

 Injury prevention

Management
 WHOLE BLOOD
◦ Contains RBC’s plasma, platelets, clotting
factors
◦ For extreme hemorrhage
 PACKED RED BLOOD CELL
◦ Use to increase the O2 carrying capacity of the
blood
◦ For anemias, blood loss due to surgery and
hemorrhage

Blood Products for Transfusion

 PLATELETS
◦ Use for client with bleeding disorder
◦ Should be administered immediately and given
rapidly, usually over 15-30 minutes
 FRESH FROZEN PLASMA
◦ Use to provide clotting factors and expand the
blood volume
◦ Does not contain RBC so crossmatching and
blood typing is not needed

Blood Products for Transfusion

 ALBUMIN
◦ Use to expand the blood volume and to treat
hypoalbuminuria
 CRYOPRECIPITATE
◦ Use for client with clotting factor deficiencies
◦ Prepared from fresh frozen plasma

Blood Products for Transfusion

1. General Precautions:
◦ T-he nurse should monitor the vital signs
before and after the fist 15 minutes of
transfusion
◦ R-eturn the blood to the blood bank if the
blood is not infused within 30 minutes
◦ A-dministration set should be changed every
4-6 hours
◦ N-o solution other than NSS can be used

Steps in Blood Transfusion

General Precaurions: (cont.)
◦ S-hould be administered within 20-30 minutes
upon arrival from the blood bank
◦ F-ollow standard policies on blood transfusion
of the agency
◦ U-nlicensed assistant personnel CANNOT
perform blood transfusion
◦ S-hould not incorporate any medication
◦ E-xpiration date, x-matching & blood typing
should be checked

Steps in Blood Transfusion

2. Prepare the client
◦ 2 RN should verify the DR’s order, client’s
identity, blood product to be administered
3. Administration of the Transfusion
◦ Start an IV line using a Y type BT set and G18 or
G19, and infuse NSS
◦ Prepare the blood bag by inverting the bag
gently to mix the cells with plasma
◦ Establish the BT and adjust flow rate slowly for
15 min at 20 gtts/min
◦ Monitor client foe possible BT complication
◦ Proper documentation

Steps in Blood Transfusion

 H-yperkalemia
◦ Due to RBC hemolysis
◦ Slow down the rate of transfusion & notify MD

 H-ypocalcemia
◦ Due to calcium excretion
◦ Slow down the rate of transfusion & notify MD

Complication of BT &
Management
 I-ron Overload
◦ Due to increase iron coming from the newly
infused RBC
◦ Administer Desferal IV or subcutaneously as
prescribed

 T-ransmission of Disease from the Donor

◦ Common diseases are Hepatitis B&C, HIV
◦ Treat the disease condition as MD’s Rx

Complication of BT &
Management
 T-ransfusion overload
◦ Due to rapid BT rate
◦ Slow down the rate of transfusion, elevate the
head of the bed & notify MD

 S-epticemia
◦ Due to contaminated blood products
◦ Notify MD

Complication of BT &
Management
 T-ransfusion Reaction
◦ Signs & Symptoms:
 R-ashes & hives
 E-xcessive perspiration (diaphoresis)
 A-pprehension
 C-hills & fever, Cephalgia, Chest Pain, Cyanosis
 T-ingling & numbness, Tachycardia
 I-tchiness
 O-liguria
 N-ausea & Vomiting

Complication of BT &
Management
 Stop the infusion and remove the blood
tubing
 Keep the vein open with NSS
 Send a sample of the client’s blood, urine
and the remaining blood to the laboratory
 Notify the MD
 Monitor the vital signs

Nursing Implications
THANK YOU