UNIT XI: HEMATOLOGICAL

DISORDERS (5 hrs)
Anemia
Thalassemia
Idiopathic Thrombocytopenic Purpura
Leukemia
Hodgkin’s and non hodgkin’s lymphoma
Hemophilia
ANEMIA
Normal Hemoglobin Level (WHO)

• 6 month to 6 year 11g/dl

• 6 year to 14 year 12g/dl

• Above 14 year in males- 13g/dl

Females-12g/dl
 NORMAL HEMOGLOBIN LEVEL
• 1-3 days: mean 18.5 g/dL

• 1 week: mean 17.5 g/dL

• 2 weeks: mean 16.5 g/dL

• 1 month: mean 14.0 g/dL

• 2-6 months: mean 11.5 g/dL

• 0.5-2 years: mean 12.0 g/dL

• 2-6 years: mean 12.5 g/dL

• 6-12 years: mean 13.5 g/dL

• 12-18 years female: mean 14.0 g/dL

• 12-18 years male: mean 14.5 g/dL

ANAEMIA
Anemia is defined as a reduction in the number of
RBC’s, the quality of hemoglobin and the volume of
packed red cells to below normal levels for age
resulting in diminished oxygen carrying capacity
of the blood to the tissues.
Etiology
Inadequate production of RBC or RBC
components
a. Nutritional deficiency
b. Inhibition of bone marrow
Increased destruction of RBC
Enzyme deficiency, drugs, chemicals,
infections, burns
Excessive loss of RBC
Acute and chronic hemorrhage
Classification
 Based on Etiology

1. Anemia due to excessive blood loss

2. Anemia due to inadequate production

3. Anemia due to increased destruction

1.Anemia due to Excessive Blood loss
I. Acute post hemorrhagic

II. Chronic post hemorrhagic

2.Anemia due to impaired red cell production
A. Deficiency of substances necessary for erythropoiesis
I. Iron deficiency anemia
II. Vitamin B 12 and folate deficiency-Megaloblastic
Anemia
B. Disturbance of proliferation and differentiation of
stem cells
I. Aplastic Anemia
C. Disturbance of bone marrow function or due to systemic
disease
I. Anemia due to infection
II. Anemia in renal disease
III.Anemia in liver disease
IV. Anemia in disseminated malignancies
V. Anemia in endocrinopathies
A.Due to bone marrow depression
I. Leukemia
II. Myelosclerosis
III.Multiple myeloma
B. Congenital anemia
I. Sickle cell anemia
II. Congenital dyserythropoietic anemia
3. Anemia due to Increased Destruction of RBC
A. Anemia due to intra corpuscular defect
I. Sickle cell anemia
II. Thalassemia
B. Anemia due to extra corpuscular defect
I. Hemolytic disease of newborn
II. Effect of toxic drugs- Primaquin,
phenytoin
III.Effect of venoms or poisons-lead
IV. Thermal injuries or burns
V. Transfusion reactions
 Based on Morphology

1. Normocytic Normochromic

2. Normocytic Hypochromic

3. Microcytic Normochromic

4. Microcytic Hypochromic

5. Macrocytic Normochromic

6. Macrocytic Hypochromic
WHO Grading

Mild: Hb level between 10gm/dl and cut off

point

Moderate: Between 7gm/dl and 10gm/dl

Severe: below 7 gm/dl

Pathophysiology
Due to etiological Causes

Decreased amount of RBC in Blood

Decrease in Oxygen carrying capacity of blood

Hypoxemia
Clinical Manifestations
• Easy fatigability
• Tiredness
• Irritability
• Lack of concentration
• Head ache
• Anorexia
• Pallor
• Skin, Nails, Conjunctiva and Mucous appears pale
• Dry and Scaly skin
• Nail- flattened, brittle with spoon shaped

(koilonichia)
• Hair- Dry listless and may easily pull off

• Decreased peripheral pulse

• Tachycardia

• Tachypnea

• Cardiomegaly- murmurs

• Splenomegaly
 Diagnosis
 Clinical Features
 CBC
RBC, Hemoglobin, Hematocrit
Mean Corpuscular Volume
Mean Corpuscular Hemoglobin
Reticulocyte
 Peripheral smear- Morphological character
 Stool Examination
 Urine- haematuria
 Bone Marrow Examination
Management
 Supportive Management:
I. Oxygen Administration
II. Blood Transfusion
III.IV Fluids and Colloids
IV. Bed Rest
 Specific Management:
Depending on type of anemia
IRON DEFICIENCY ANEMIA
Due to inadequate dietary supply of iron or loss
of iron
Incidence:
 Common in preschool and adolescent children due to
their rapid growth rate and poor eating habits
 Common in preterm due to reduced fetal iron supply
 Most prevalent nutritional disorder
 Common in low socioeconomic status
 Etiology
 Low iron stores

 Reduced iron intake

 Excessive loss of iron from the

body
 Decreased iron absorption

 Increased iron demands

 Defective iron metabolism

 Pathophysiology
Due to etiological factor

Deficient iron stores

Reduction in the production of hemoglobin

Reduced oxygen carrying capacity of the blood

 Clinical Manifestation
Pallor, Irritability, fatigue, Listless, Weakness,
Anorexia, Failure to thrive
Specific
Hair Fall
Koilonichia
Splenomegaly
Cardiomegaly
Widening Of Cranial Sutures
Pica
Clinical Features
 Diagnosis
History and physical examination
Peripheral Smear
Microcytic hypochromic RBC
CBC
 Low hemoglobin
 Reduced RBC
 PCV,MCH,MCHC,MCV are low
 Serum Fe is low
 Total Iron Binding Capacity is high
Stool – Helminthes , occult blood (Guaiac tests)
Urine- RBC, Pus cells, hematuria
 Management
 Underlying cause should be

treated
• Supplementation of iron
 Oral Iron therapy: ferrous sulphate, Ferrous Gluconate
Dose: 3-6mg/kg/day in divided dose
 Parenteral therapy: indicated only when oral is not
tolerated
Iron Dextran is used, which contains 50mg elemental
iron per ml
Route: IM using Z tract method or IV by dissolving in
250-500ml NS over 6-8hours
Dose = Weight in Kg X Hb deficit(g/dl) X 4
Blood Transfusion

 Indicated when Hb below 4gm/dl

 Only packed cells should be given slowly

 Frusemide 1-2mg/kg intravenously should be given to

prevent circulatory overload

• Dietary management:
 Iron rich foods
 Iron fortified formula
 Restriction on milk intake
 Older infants and toddlers-finger foods
such as thinly sliced meats
 Adolescents –foods with high iron
content and vit-c
 Folic acid-converts iron from ferritin
to Hb
 NURSING MANAGEMENT OF CHILD WHO IS ON
IRON THERAPY

Parental education on following aspects

I. Proper administration of iron

supplements
II. Side effects of iron therapy

III.Improving dietary iron intake

• Proper administration of iron supplements
Given between meals, because presence of
HCl facilitate iron absorption
Should not administer with milk or dairy
products containing calcium, which reduce
iron absorption
 Antacids inhibits iron absorption
Administer with vit C which helps iron to
reduce to its most soluble form
Liquid form of iron can stain the teeth –
dropper, syringe or straw can be used; or
rinse the mouth or brush the teeth after
drinking
• Side Effects of Iron therapy
 Abdominal cramps, Vomiting,
Diarrhea or Constipation, Black
stools, GI discomfort, Foul taste
• Improving Dietary intake
 animal and plant foods: meat, liver,
kidney, egg yolk, green leafy
vegetables and fruits like apple
 Foods should be prepared in utensils
made up of iron
 PREVENTION
Adequate antenatal care for prevention of
maternal anemia
Prevention of preterm, LBW and control of
infection
Exclusive breast feeding till 4-6 month
Iron fortified milk formulas should be given for
non breastfed infants
De worming periodically
Iron folic acid supplementation
Improve dietary intake
Wear foot wears while playing
Improve living standards
 Nursing assessment
Screening for anemia is performed between 9
months and 1 year
Second screening at 15-18 months
Premature infants screened at 4 months
Follow up screening for all children at 2 and 3
years
Screening during adolescent period is
recommended
MEGALOBLASTIC ANEMIA
Results from deficiency of folic acid

or vitamin B12 or both impairs the

maturation of erythrocytes leading to
formation of abnormally large
erythrocytes known as megaloblast
 Etiology
Decreased dietary intake- exclusively
breast fed, infants of mothers who are
vegetarian, anemic and malnourished
Infant with chronic diarrhea,
malabsorption or recurrent infection
Drugs- Phenytoin, pyrimethamin,
Chemotherapy
Increased demand
Excessive urinary folate loss
Clinical features
• Pale, sick, irritable, severe anorexia and FTT
• Increased pigmentation of back, nose and hands
• Older children- sore red tongue, angular
stomatitis, glossitis, diarrhea
• Neurological Manifestation such as numbness,
paresthesia, weakness, ataxia and diminished
reflexes
 DIAGNOSIS
•Peripheral blood smear: Anisocytosis,

poikilocytosis with Macrocytic Normochromic RBC

•Serum vitamin b12 and folic acid assay:

 Vita B12 < 100pg/ml (Normal 200-900 pg/ml)

 Folate level < 4ng/ml (Normal: 5-21 ng/mL)

• Bone Marrow- Increased Megaloblast
 Management
Administration of deficient folic acid and Vitamin

B12
Vit B12- 1 microgram/day

Folic Acid- 2-5mg/kg/day

Encourage folate Diet- fresh leafy vegetables,

Legumes, Nuts, Meat

 APLASTIC ANEMIA
 It is a deficiency of the blood cells that results from

failure of the bone marrow to produce adequate

numbers of circulating blood cells.
 Caused by bone marrow depression and involves the

blood elements resulting in pancytopenia

 Etiology
 Autoimmune Suppression
 Toxic Agents: Industrial and household chemicals
 Pharmacologic Agents: antibiotic, Anti inflammatory,
Anticonvulsants, Antimalarial, Oral hypoglycemic agent
 Infection with HPV, hepatitis
 Irradiation
 Infiltration and replacement of myeloid elements in
leukemia and lymphomas
 Idiopathic
Pathophysiology
o Bone marrow hematopoietic failure

o Pancytopenia

o Marked reduction in stem cells and the

replacement of red bone marrow by fat
 Clinical Manifestation
Increased bruising caused by decreased
platelet
Vulnerable to infections
Pallor, Weakness, breathing difficulty
Impaired Growth and development
 Types

Congenital (primary)-Fanconi’s

anemia

Acquired- idiopathic/due to

Secondary causes
Acquired aplastic anemia
Severe: less than 25% bone marrow cellularity

with atleast two of the following findings:

 Absolute granulocyte count < 500/mm³

 Platelet count < 20,000/mm³

 Absolute reticulocyte count < 40,000/mm³

Moderate
 Diagnosis:
History and clinical features

CBC- Pancytopenia, decreased Hb and Hematocrit

Serum iron –elevated

Bone marrow biopsy: shows hypocellular marrow -

fatty bone marrow instead of red bone marrow

MANAGEMENT
Goals:
 To provide symptomatic treatment

 Restore bone marrow function

 Replace pathological bone marrow

with normal tissue

• Symptomatic treatment
If platelet count < 10,000- Platelet transfusion
Severe Anemia- packed cells
Inections - Antibiotics
• Restoration of bone marrow function
Androgenic Steroids and Corticosteroids: Testosterone
converts hypo cellular bone marrow into nearly normal
bone marrow
High doses of dexamethasone or Immunosuppressive
therapy: Antilymphocyte Globulin (ALT), Antithymocyte
Globulin (ATG)
• Bone Marrow transplantation
 Nursing management
 Preventing bleeding
 blood transfusion
 Preventing infection
 Encouraging mobility
 Health education
 Providing emotional support
SICKLE CELL ANAEMIA

It is one of the group of diseases collectively

termed hemoglobinopathies, in which normal adult
Hb {HbA} is partly or completely replaced by
abnormal sickle haemoglobin {HbS}

It includes all those hereditary disorders

whose clinical, hematologic, and pathologic features
are related to the presence of HbS
 ETIOLOGY AND PATHOPHYSIOLOGY
It is autosomal recessive disorder
Although the defect is inherited, the sickling
phenomenon is not evident till later infancy because
of the presence of fetal Hb {HbF}.
 The newborn with SCA is asymptomatic because of
the protective effect of HbF. But it rapidly
decreases during the first year of life, so child is at
greater risk to develop complications
 SCA, the homozygous form of the disease (HbgSS), in
which valine, an amino acid, is substituted for glutamic acid
at the sixth position of the β chain
• Sickle cell–C disease, a heterozygous variant of
SCD (HgbSC) is characterized by the presence of both
HgbS and HgbC, in which lysine is substituted for
glutamic acid at the sixth position of the β chain
• Sickle thalassemia disease, a combination of sickle
cell trait and β-thalassemia trait (Sβthal). In the β+
(beta plus) form, some normal HbA can be produced.
In the β0 (beta zero) form, there is no ability to
produce HbA
PATHOPHYSIOLOGY:
The clinical features are primarily due to
(1) Obstruction caused by sickle RBCs
(2) Increased RBC destruction
 Abnormal adhesion and Entanglement of rigid
PATHOPHYSIOLOGY:
sickle-shaped cells with one another

Blocks micro-circulation

Vaso-occlusion

Reduced blood supply to adjacent tissues

Hypoxia

Tissue ischemia and infarction (cell death)

The effect of sickling and infarction on
organ structures occurs in following
sequence.

1. Stasis with enlargement

2. Infarction with ischemia

and destruction
3. Replacement with fibrous

tissue
 CLINICAL MANIFESTATIONS
General:
 Growth retardation
 Chronic anaemia
Delayed sexual maturation
Marked susceptibility to sepsis
Vaso-occlusive crisis:
Pain in areas involved.
Extremities: Painful swelling of hands
and feet
Abdomen: Severe pain
 Cerebrum: Stroke, Visual disturbances,
Chest: Symptoms resembling pneumonia, episodes of
pulmonary disease
Liver: Obstructive jaundice, hepatic coma
Kidney: Hematuria
Genitalia: Priapism (Painful, Constant penile erection)
Sequestration crisis:

Pooling of large amount of blood-

Hepatomegaly,

Spleenomegaly,

Circulatory collapse
 Effects of chronic vaso-occlusive phenomena:
• Heart: Cardiomegaly, Systolic murmurs,
• Lungs: Risk for infections, pulmonary
insufficiency
• Kidneys: Inability to concentrate urine,
progressive renal failure.
• Liver: Hepatomegaly, Cirrhosis
• Spleen: Spleenomegaly, susceptibility to
infection, decreased spleenic activity
• Eyes: visual disturbances sometimes progressive
retinal detachment and blindness
• Extremities: Avascular necrosis of hip or
shoulder, Skeletal deformities
• CNS: Hemiparesis, seizures
Types of crisis
1.Vaso occlusive crisis:
 Most common and painful
Sickled cell obstructing the
blood vessels causing the
occlusion ischemia and necrosis
2.Splenic sequestration crisis:
 Caused by the spleen sequestering (pooling)
large quantities of blood causing the drop in
blood volume and ultimately shock.
Acute or chronic.
 It can result in death.
3.Aplastic crisis:
 Due to the diminished RBCs production by
any triggering factors like infections.
When superimposed on the existing rapid
destruction of the cells leads to the anemia
 DIAGNOSIS
Routine hematological tests.
Sickle cell turbidity test:
for quick screening purposes in children
>6 months of age once the fetal Hb levels
have fallen
CBC-decreased Hb and Increased RBC’s
Hb electrophoresis for detecting the
homozygous and heterozygous forms of the
disease, as well as the percentages of the
various types of Hb
MANAGEMENT
The main aim of therapy is

 To prevent conditions that enhance sickling

phenomenon.

 To treat the medical emergencies.

 SUPPORTIVE AND SYMPTOMATIC
MANAGEMENT
 Rest
 Hydration: Oral and IV fluid replacement and partial
exchange transfusion if needed. Fluids to reduce the
viscosity of blood
 Electrolyte replacement, because hypoxia results in
metabolic acidosis, which also promotes sickling.
 Analgesics
 Blood replacement
 Antibiotics
Prevention and Treatment of infection
 Who are functionally asplenic or have had a
splenectomy have resultant decreased capability
to fight infection
 vit-K,125 mg,BD from 2 months -3 years,250 mg-
3-5 years
 Amoxycillin every 3 weeks
 If allergic to penicillin-erythromycin 20 mg/kg,BD
in divided doses
 Blood Cultures-if infection suspected
 Administration of pneumococcal, influenza and
meningococcal vaccines
 Transfusion of RBC’s
Improves blood and tissue oxygenation

Reduction in sickling

Other therapies:
Hydroxyurea- decreases production of

abnormal blood cells and leads to lesser

amount of pain
Hemotopoietic stem cell transplant
Nursing management
 Identifying children at risk

 Recognition and management of sickle cell

crisis
 Promoting G & D

 Prevention of complications

 Supporting the child & family

Acute pain related to tissue anoxia
(vasoocclusive episode or crisis)
 Nursing Interventions Rationales
 Discuss schedule of medication around the clock with parents. To
control pain
 Encourage high level of fluid intake. To ensure hydration
 Recognize that various analgesics, including opioids and
medication schedules, may need to be tried. To ensure satisfactory
pain relief
 Reassure child and family that analgesics, including opioids, are
medically indicated, that high doses may be needed, and that
children rarely become addicted.
 To avoid needless suffering because of unfounded fears
 Apply heat application or massage to affected area. Avoid applying
cold compresses. To prevent vasoconstriction that may enhance
sickling