HEMATOLOGIC

DISORDERS
 • The blood and the blood forming
sites, including the bone marrow
Hematologic and the reticuloendothelial
system (RES)
System • Blood
• Plasma: fluid portion of blood
• Blood cells: erythrocytes,
leukocytes, thrombocytes
• Hematopoiesis
•Adult:
Female 4-5L
Male 5-6L
 • Transport of
• Gases, nutrients, wastes
products
• Processed molecules
Functions • Regulatory molecules
• Regulation of pH and osmosis
of Blood • Maintenance of body temperature
• Protection against foreign
substances
• Clot formation
 • Liquid part of blood
• Pale yellow made up of 91% water,
Plasma 9% other

• Colloid: Liquid containing suspended

substances that don’t settle out
• Albumin: important in regulation of
water movement between tissues
and blood
• Globulins: immune system or
transport molecules
• Fibrinogen: responsible for formation
of blood clots
 Erythrocyte: RBC
Blood Cells
Leukocyte: WBC
• Neutrophil
• Monocyte
• Eosinophil
• Basophil
• Lymphocyte: T lymphocyte and B
lymphocyte

Thrombocyte: platlet
 • Hematopoiesis or hemopoiesis
• Process of blood cell production
Production of • Fetus: liver, thymus, spleen, lymph nodes,
red bone marrow
Formed • After birth: red bone marrow, for WBC in
some lymphatic tissues
Elements
• Stem cells (hemocytoblast): All formed
elements derived from single population
• Proeryhtroblasts: develop into red blood
cells
• Megakaryobalsts: develop into platelets
Erythrocytes
Structure
• Biconcave, anucleate

Components
• Hemoglobin (makes it red in color)
• Lipids, ATP, carbonic anhydrase

Function
• Transport oxygen from lungs to tissues
and carbon dioxide from tissues to
lungs
Fate of Erythrocytes
• Unable to divide, grow or synthesize proteins
• Wear out in 100 to 120 days
• Removed by phagocytes in the spleen or liver
• New RBCs made by stem cells in bone marrow
• Production increases when oxygen level decrease or
during pregnancy
Hemoglobin

• Consists of
• 4 globin molecules: Transport carbon
dioxide (carbonic anhydrase
involved), nitric oxide
• 2 alpha and 2 beta globin chains
• 4 heme molecules: Transport oxygen
• Each heme contains 1 iron
• Iron is required for oxygen
transport
• Hemoglobin + oxygen = bright red
• Hemoglobin with no oxygen = darker
red
 Erythrocytes: Levels in Blood

01 02 03
Live only for four Average RBC count: Outnumber white
(4) months or 120 • Males: 5.4 million/mm3 blood cells 1000:1
days • Females: 4.8 million/
mm3
 Erythropoiesis
• Happens in red bone marrow
• Components required:
• Precursor cells:
PROERYTHROBLAST
• Proper microenvironment
• Adequate supplies of iron,
vitamin B12, folic acid,
protein, pyridoxine and
traces of copper
• Erythropoietin:
• Hormone to stimulate RBC
production
• During cell division, the
process requires B vitamins
folate and B12, necessary
for synthesis of DNA.
• Iron is required for
production of hemoglobin
• RBC production is triggered
by low blood oxygen levels.
 Lymph capillaries

• Carries lymph to subclavian

vein in the chest.
Lymphatics
Lymph nodes

• Small rounded structures that

filter bacteria and foreign
particles
 Reduction below normal in the # of
RBC, the quantity of hemoglobin
and volume of RBC (hematocrit)

ANEMIA
Result from
↓ RBC ↑ RBC Acute or chronic
production destruction blood loss
Three broad categories
1. Loss of RBC – occurs with
bleeding
2. Decreased RBC
production
• IDA
• FADA
• Thalassemia
3. Increased RBC
destruction
Iron Deficiency Anemia
 • Iron Deficiency Anemia
• Dietary intake of iron is
inadequate to produce
hemoglobin
Hypoproliferative • Most common type of
Anemia anemia
• May occur with removal of
the duodenum
• Associated with chronic
blood loss.
Etiologic Factors
1. Bleeding – the most common cause
2. Mal-absorption
3. Malnutrition
4. Alcoholism
Pathophysiology
• The body storage of iron
decrease, leading to depletion of
hemoglobin synthesis
• The oxygen carrying capacity of
hemoglobin is reduced → tissue
hypoxia
 Assessment Findings

Pallor of the skin

Weakness and
and mucous General malaise Pica
fatigue
membrane

Smooth and
Brittle nails Angular cheilosis
sore tongue
 Laboratory Findings

1.CBC
• Low levels of Hct, Hgb and RBC count
2. Low serum iron, low ferritin
3. Bone marrow aspiration – MOST definitive
 Iron replacement
Medical
Management
Blood transfusion
Nursing Management

1. Provide iron-rich foods

• Organ meats (liver)
• Beans
• Leafy green vegetables
• Raisins and molasses
2. Administer iron
• Oral preparations tablets – Fe fumarate, sulfate and gluconate
• Oral preparations – liquid
• It stains teeth – drink it with a straw
• Advise to take iron ONE hour before meals
• Take it with vitamin C
• Monitor for adverse reaction like pyrosis (heartburn),
constipation, diarrhea, dark stool (stool may turn blackish-
dark in color)
• Continue taking it for several months even with normal iron
level
• Advise to eat high-fiber diet and to ↑ fluid intake to
counteract constipation
• IM preparation
• Administer DEEP IM
• Include 0.5ml air in syringe to clear iron
from the needle
• Use the Z track method
• Avoid vigorous rubbing
• Can cause local pain and staining

• Note
• Iron should be taken for 2-3 months after
Hgb level returns to normal
3. Administer packed
red blood cell
transfusion if patient is
symptomatic
THALASSEMIA
 • Normally hemoglobin contains 4 globin
chains
• Decreased production of hemoglobin
due to abnormal hemoglobin synthesis
• Reduced production or no production of
1 of the globin chains that make up
hemoglobin
THALASSEMIA • Microcytic and hypochromic
• Autosomal recessive genetic disorder
common among Mediterranean people
• Chronic bone marrow hyperplasia
• Altered globin synthesis of hemoglobin
• Treatment causes chronic iron toxicity
• Thalassemia minor
• Has 1 thalassemic and 1 normal
gene with mild clinical
manifestations
• Requires no treatment

• Thalassemia major
• Has 2 thalassemic genes causing
severe conditions
 Autosomal
Recessive Pattern
of Inheritance
 • Skin: pale/jaundiced
• Splenomegaly and
hepatomegaly
Assessment • Thickened cranium and
maxillary sinus space
from bone marrow
hyperplasia
Diagnostics

• CBC
• Anemia lower mean
corpuscular volume
• Hemoglobin
electrophoresis
Nursing Management

Administer BT and chelation therapy (remove heavy

Administer metals) to reduce iron overload

Monitor for transfusion reactions or diseases acquired

Monitor through transfusions.

Instruct Instruct about treatment, medications and physical

about energy conservation techniques

Strengthen Strengthen client support and family systems

APLASTIC
ANEMIA
APLASTIC ANEMIA

• Life-threatening stem cell disorder with many possible

etiological mechanisms
• Decreased number of RBC as well as WBC and platelets
• Characterized as:
• Hypoplastic (incomplete development of a tissue or organ)
• Fatty bone marrow
• Pancytopenia (reduction in # of RBC, WBC and platelets
 • Environmental toxins –
pesticides, benzene
• Certain drugs
Causative • Chemotherapeutic agents
• Chloramphenicol
Factors • Phenothiazines
• Sulfonamides
• Heavy metals
• Radiation
 Toxins cause a direct bone
marrow depression
↓
Pathophysiology Acellular bone marrow
↓
Decreased production of blood
elements
Assessment Findings
• Fatigue
• Pallor
• Dyspnea
• Bruising
• Splenomegaly
• Retinal hemorrhages
 • CBC
• Decreased blood cell
numbers
Laboratory
Findings • Bone marrow aspiration
confirms the anemia –
hypoplastic or acellular marrow
replaced by fats.
• Bone marrow
transplantation
• Immunosuppress
ant drugs
• Rarely, steroids Medical
• Blood transfusion. Management
 Nursing Assessment

Assess for signs of bleeding and infection

Pancytopenia plan of care to prevent complications like infection and

bleeding
• Private room
• Strict handwashing
• Minimizing invasive procedures.

Provide client and family with support for lengthy hospitalization and
treatment
Megaloblastic Anemias
 • Defective erythrocytes
(megaloblast) structure
Megaloblastic caused by impaired
Anemias DNA synthesis caused
by B12 or folate
deficiency.
Folic Acid Deficiency
• Folate is required for DNA synthesis in
erythrocyte formation
• Without neurologic involvement

Causative factors:
1. Alcoholism
2. Mal-absorption
3. Diet deficient in uncooked vegetables
4. Use of oral contraceptives.
 Decreased folic acid
↓
Impaired DNA synthesis in the bone marrow
Pathophysiology ↓
Impaired RBC development
Impaired nuclear maturation but cytoplasmic
maturation continues
↓
Large size
 • Poor nutrition
• Alcohol abuse
• Anorexia
• Impaired absorption in the small intestine
Assessment • Undergoing hemodialysis (folate is dialyzable)
• Certain drugs can block folate absorption
• Oral contraceptives
• Antiseizure drugs (phenytoin)
• Antibiotics
Diagnosis

↓ RBC, Hgb, Hct and

CBC
↑MCV

Serum folate levels are low

Serum cobalamin is normal

 Promote compliance with replacement
Promote therapy (1-5 mg oral folate/day)

Nursing Administer Administer prenatal vitamins in pregnancy

Management

Instruct on food high in folate such as

Instruct on leafy greens, liver, citrus fruits, nuts and
grains.
PERNICIOUS
ANEMIA
Pernicious Anemia

Normal Condition:
Parietal cells of stomach
secreted intrinsic factor
to absorb ingested
cobalamin (Vit. B12)
• Vitamin B12 Deficiency (Pernicious Anemia)
• Lack of intrinsic factor (needed for B12
absorption)
• Common among Northern European ancestry
over age of 40 and young African.

Causative factors:
• Strict vegetarian diet
• Gastrointestinal malabsorption
• Crohn’s disease
• Years of Gastritis
• Gastrectomy
 • Peripheral blood smear – shows giant
RBCs, WBCs with giant hyper
segmented nuclei
• Very high MCV
• Schilling’s test
Laboratory • Radioactive vit B12 malabsorption is
Findings measured by small amount of secreted in
urine
• When vit B12 is administered with
gastric/ if parenterally, its absence in
urine will diagnose pernicious anemia
• Intrinsic factor antibody test
 • Vitamin supplementation
• Folic acid 1 mg daily

• Diet supplementation
Medical • Vegetarians should have
vitamin intake
Management
• Lifetime monthly injection of
IM Vit B12
• Without cobalamin
replacement, patient can die in
1-3 years.
• Since Pernicious Anemia
results from an inability
to absorb cobalamin,
dietary intake of the
vitamin is not a
treatment option, nor is
bone marrow transplant.
 • Monitor patient
• Provide assistance in
ambulation
Nursing
• Oral care for tongue
Management
sore
• Explain the need for
lifetime injection of vit
B12
ANEMIA FROM
CHRONIC
DISEASE
 • Decrease in erythrocyte
Anemia precursor that occurs in some
chronic conditions.
from
Chronic • End-stage renal failure
• Chronic liver disease
Disease • Alcohol abuse
• Hypothyroidism
 Clinical
Manifestations
All assessment findings for
anemia apply.
Diagnosis

CBC Ferritin

• Iron stores may be

high in contrast to
IDA
Nursing Management

• Facilitate diagnosis and treatment if underlying,

contributory condition.
• Inform that this type of anemia does not respond
to folic acid, iron or vitamin B12.
• Erythropoietin therapy is administered to a client
with anemia related to renal failure.
 ANEMIA CAUSED BY
INCREASED RBC DESTRUCTION
 2 Sites of Hemolysis
• Intravascular Destruction
• Occurs within the
circulation

• Extravascular Destruction
• Occurs in liver, spleen or
bone marrow
 • Intrinsic Hemolytic Anemias
• Usually hereditary
• Defects in RBC themselves
Classifications • Abnormal Hgb = sickle cell
of Hemolytic anemia
Anemias • Enzyme deficiencies =
G6PD
• Cell membrane
abnormalities
• Extrinsic Hemolytic Anemias
• Normal RBCs are damaged by external factors
• Cause:
• Antibodies, toxins
• Mechanical injury (prosthetic heart valves)
• Dialysis
• Transfusion reaction
• Trapping of cells within the liver and spleen
Assessment

• Jaundice due to bilirubin levels from destruction of the

RBC
• Enlarged liver and spleen due to sequestration of
defective RNC in these organs
• Abdominal pain
• Melena
• Diarrhea
• hematuria
HEMOLYTIC ANEMIA: Sickle Cell
Sickle Cell Anemia

• A severe chronic incurable hemolytic anemia that

results from inheritance of the sickle hemoglobin
gene.
• Produces specific mutant form of beta-globin
• Hypoxia-induced change in RBCs
• Associated with vascular occlusion and tissue
infarction
• Anemia is caused by accelerated breakdown of
abnormal RBC
 Causative factor
Genetic inheritance of the sickle
gene – HbS gene
 Pathophysiology

Decreased O2, cold,

vasoconstriction can
precipitate sickling
process
 Factors
↓
Cause defective hemoglobin to acquire a
rigid, crystal-like C-shaped configuration
↓
Sickled RBCs will adhere to endothelium
↓
Pile up and plug the vessels
↓
Ischemia results
↓
Pain, swelling and fever
 • Jaundice
• Enlarged skull and facial
bones
Assessment • Tachycardia, murmurs
Findings and cardiomegaly
• Primary sites of
thrombotic occlusion:
spleen, lungs and CNS
• Chest pain, dyspnea
 • Sickle cell crises
• Results from tissue hypoxia
and necrosis
Assessment
• Acute chest syndrome
Findings • Manifested by a rapidly
falling hemoglobin level,
tachycardia, fever and
chest infiltrates in the CXR
• During the sickling crisis, the sickling cells clog small
capillaries, and the resulting hemostasis promotes a self-
perpetuating cycle of local hypoxia, deoxygenation of more
erythrocytes and more sickling.

• Medical Management:
• Administration of large doses of narcotic analgesics since pain last
4-6 days
• Bone marrow transplant
• Hydroxyurea
• Long term RBC transfusion
 Nursing Management

• Manage the pain

• Support and elevate acutely
inflamed joint
• Relaxation techniques to reduce
metabolic needs
• Analgesics
• Prevent and manage infection.
• Monitor status of patient
• Initiate prompt antibiotic therapy
• Promote coping skills
• Provide accurate information
• Allow patient to verbalize her
concerns about medication,
prognosis and future pregnancy
• Monitor and prevent potential complications.
• Provide always adequate hydration
• Avoid cold, temperature that may cause vasoconstriction
• Leg ulcer
• Aseptic technique
• Priapism
• Sudden painful erection
• Instruct patient to empty bladder, then take a warm
bath
• Maintain F&E balance to reduce blood viscosity.
 GLUCOSE 6-PHOSPHATE
DEHYDROGENASE (G6PD) DEFICIENCY
 Glucose-6-phosphate
dehydrogenase

G6PD
Results from lack of an RBC
enzyme that leads to RBC
damage when metabolic needs
of RBCs are increased.
 G6PD

• A group of X-linked familial hemolytic mutations of the

gene
• Deficiency predisposes to oxidative denaturation of
hemoglobin, with resultant red cell injury and lysis.
• Hemolysis occurs as the result of oxidative stress generated
by either an infection or exposure to certain drugs.
• More common among males of Mediterranean or African
descent.
Assessment
• Jaundice
• Headache
• Dizziness
• Easy fatigability
 Use of G6PD assay or screening
test

Diagnostic
Quantification of G6PD levels
during nonhemolytic phase
 01 02 03
Nursing Provide periods Provide Administer
antibiotics and
of rest adequate
Management hydration
other treatment as
ordered to
minimize
complications.
Polycythemia Vera
 Refers to an
increase volume
of RBCs
Polycythemia
The hematocrit is
elevated to more
than 55%.
 • A proliferative disorder
which the myeloid stem
Primary cells become uncontrolled.
Polycythemia
• Neoplastic disorder where
there is an increased in 3
types of blood cells.
• Causative factor: unknown
 • The stem cells grow uncontrollably
• The bone marrow becomes
hypercellular and all the blood cells
are increased in number.
• The spleen resumes its function of
Pathophysiology hematopoiesis and enlarges
• Blood becomes thick and viscous
causing sluggish circulation
• Overtime, the bone marrow
becomes fibrotic.
 Skin is ruddy, pruritic due to histamine
release

Splenomegaly in primary PV only

Assessment Headache, tinnitus

Findings
Dizziness, blurred vision

Angina, intermittent claudication, dyspnea

and thrombophlebitis.
 CBC – shows elevated
RBC mass

Laboratory Normal oxygen

Findings saturation

Elevated WBC and

platelets
 Increased risk for
• Thrombophlebitis
• Due to hypervolemia and
hyperviscosity
• Treatment: active/passive leg exercise
Complications and ambulation
• CVA
• MI

Bleeding due to dysfunctional

blood cells
Medical Management
• To reduce the high blood cell mass –
PHLEBOTOMY
• Allopurinol
• Dipyridamole
• Chemotherapy to suppress bone marrow.
• Primary role of the nurse is
EDUCATOR.
• Assist in weekly phlebotomy
Nursing
• Advise to avoid alcohol and
aspirin
Management
• Advise tepid sponge bath or
cool water to manage
pruritus.
LEUKEMIA
 • Malignant disorders of blood
forming cells.
• Characterized by
UNCONTROLLED proliferation
Leukemia of WBC in the bone marrow-
replacing marrow elements
• The WBC can also proliferate in
the liver, spleen and lymph
nodes.
Leukemia
• Named after the specific lines
of blood cells affected
primarily
• Myeloid
• Lymphoid
• Monocytic
 The leukemias are named also
according to the maturation of cells.

ACUTE
Leukemia • The cells are primarily immature.

CHRONIC
• The cells are primarily mature of
differentiated
 ACUTE ACUTE
myelocytic lymphocytic
leukemia leukemia

CHRONIC CHRONIC
myelocytic lymphocytic
leukemia leukemia
 Unknown

Probably exposure to radiation

Etiologic Chemical agents

Factors
Infectious agents

Genetic
 Uncontrolled proliferation of
immature cells
↓
Pathophysiology Suppresses bone marrow
(Acute function
Leukemia)
↓
Severe anemia,
thrombocytopenia and
granulocytopenia
 Uncontrolled proliferation
of differentiated cells
Pathophysiology ↓
(CHRONIC Slow suppression of bone
Leukemia) marrow function
↓
Milder symptoms
Assessment Findings
• Acute Leukemia
• Pallor
• Fatigue
• Dyspnea
• Hemorrhages
• Organomegaly
• Headache
• Vomiting
• Chronic Leukemia
• Less severe symptoms
• organomegaly
 • Peripheral WBC count
varies widely
• Bone marrow aspiration
Laboratory biopsy reveals a large
Findings percentage of immature
cells – BLASTS
• Erythrocytes and
platelets are decreased.
 • Chemotherapy
Medical
Management • Bone marrow
transplantation
Nursing Management
• Manage and prevent infection
• Monitor temperature
• Assess for signs of infection
• Be alert if the neutrophil count drops
below 1000 cells/mm3
01 02 03
Skin integrity Provide pain relief Provide information
as to therapy –
chemotherapy and
bone marrow
transplantation.