HEMATOLOGY

FANER, Ned Denebe

LACANILAO, Sunshine
NUCUM, Billie Kim
PAGADUAN, Maribec
PUA, Monalisa
 BLOOD
Small
Monocyte lymphocyte

Platelets Neutrophil

Small Eosinophil
lymphocyte
Erythrocyte
Neutrophil
Young (band)
Large neutrophil
Lymphocyte Monocyte
Neutrophil
Basophil
 Red Blood Cells
 small,biconcave disks that lack a nucleus
when mature. 4 to 6 million red blood cells
per mm3 of whole blood.
 Red blood cells transport oxygen, and
each contains about 200 million molecules
of hemoglobin, the respiratory pigment.
 Make ATP by anaerobic metabolism
HEMOGLOBIN

The cytoplasm of an RBC consists mainly of a

33%solution of hemoglobin (Hb), the red pigment
that gives the RBC its color and name.

lungs
 Hb + O2 HbO2
tissues
HEMOGLOBIN
 Hemoglobin consists of four protein
chains.
Each chain is
conjugated with a
β α nonprotein moiety
called the heme
group, which binds
oxygen to a ferrous
α β ion
(Fe2) at its center
 Hypoxemia RBC PRODUCTION AND
(inadequate O2 transport REGULATION

Sensed by The kidneys release

liver and Increased increased
kidneys O2 transport amounts of
erythropoietin whenever
the oxygen capacity of
the blood is
reduced. Erythropoietin
stimulates the red bone
Increased marrow to speed up its
Secretion of
RBC count production of red blood
erythropoietin
cells, which carry
oxygen. Once the
oxygen-carrying
Accelerated capacity of the blood is
erythropoiesis sufficient to support
normal cellular activity,
the
kidneys cut back on their
Stimulation of production of
red bone marrow erythropoietin.
 ANEMIA Impaired erythropoietin
production
Decreased
erythropoietin Impaired cellular response to
effect
erythropoietin (e.g. anemia of
chronic diseases)

↓ proliferation
By external agents, physical
or chemical (e.g. ionizing
radiation, marrow toxins
Marrow
damage Hereditary or acquired
or defect aplastic anemia

Intrinsic marrow replacement

(e.g. myelofibrosis
 ANEMIA
Megaloblastic Vit B12 deficiency

macrocytic Folate deficiency

Maturation
defect
Iron deficiency and the
anemia of chronic disease
Microcytic
Impaired globin chain
(hypochromic synthesis
(thalassemias)
Impaired porphyrin
synthesis
 ANEMIA Membrane defects (e.g.
hereditary
Phagocytosis by spherocytosis)
reticuloendothelial
cells Heinz body associate
(e.g. G6PD deficiency)
Hemoglobin discorders
(e.g. sickle cell)
Accelerated Red cell
fragmentation DIC
Hemolysis
syndromes Vasculitis
syndromes
Sickle cell

Intravascular Osmotic and

hemolysis physical injury
 ANEMIAS
Type Morphologic Causes Underlying
characteristics Pathophysiology
Microcytic: Microcytic; Inadequate Insufficient iron stores lead to
Iron hypochromic diet a depleted RBC mass with
deficiency; Blood loss, subnormal hgb conc, and in
chronic blood chronic turn, subnormal O2 carrying
loss capacity of the blood

Macrocytic or Macrocytic with Inadequate Vit B12 deficiency Inhibits

megaloblastic; variation in size,
pernicious or shape of RBCs
folic acid
diet, lack of cell growth; deformed RBCs
intrinsic with poor O2 carrying capacity
factor for Neuro damage occurs bec
pernicious VB12 impairs myelin formation
anemia, Deficiency of folic acid results
impaired in inhibits cell growth, which
absorption have shortened life span
 ANEMIAS
Type Morphologic Causes Underlying
Characteristics Pathophysiology

Aplastic Normocytic, drug toxicity, Damage of

normochromic RBCs, genetic failure, destroyed stem cells
depletion of radiation, inhibit blood cell
leukocytes and chemicals, production
platelets infections
Hemolytic Normocytic, Mechanical injury, Reduced RBC
normochromic, inc RBC antigen- survival
number of antibody reaction,
reticulocytes chemical reactions

Post Normocytic, Internal or external Reduced circulating

hemorrhagic; normochromic, inc hemorrhage blood volume
acute number of
hemorrhage reticulocytes within
48-72 h
 POLYCYTHEMIA VERA
 Uncontrolled and rapid cellular
reproduction and maturation cause
proliferation or hyperplasia of all bone
marrow cells (panmyelosis)
 ↑ RBC mass, ↑ blood viscosity, inhibits
blood flow to microcirculation
 ↓ blood flow and thrombocytosis set the
stage for intravascular thrombosis
SICKLE CELL
ANEMIA
OVERVIEW
 Sickle-celldisease is a general term for a
group of genetic disorders caused by
sickle hemoglobin (Hgb S or Hb S).
 Erythrocytes become elongated and
crescent shaped (sickled)
 removed from the circulation and destroyed at
increased rates, leading to anemia.
OVERVIEW
 An autosomal
recessive inherited
defect
 The disease is
chronic and lifelong.
 Lifespan is often
shortened with
sufferers living to an
average of 40 years.
OVERVIEW
 The polymerization of deoxygenated HbS is the
primary indispensable event in the molecular
pathogenesis of sickle cell disease
 HbS polymerization is associated with increased
red cell density (dense erythrocytes) as well as
red cell membrane damage favoring the
generation of distorted rigid sickle cells and
contributing to vaso-occlusion and premature
red cell destruction (hemolytic anemia).
OVERVIEW
 The gene defect is a known mutation of a
single nucleotide polymorphism (SNP) (A
to T) of the β-globin gene, which results in
glutamic acid to be substituted by valine at
position 6.
 GAG to GUG codon mutation = LEADING
TO HbS FORMATION
OVERVIEW
 Fetalhemoglobin contains a gamma, not
a beta chain, the disease usually will not
result in clinical symptoms until the child’s
hemoglobin changes from the fetal to the
adult form at approximately 6 months.
Sickle-Cell Trait
 Bothparents with the disease will have
both normal adult and hemoglobin S and
be carriers (heterozygous) of the SICKLE-
CELL TRAIT.
 25% - 50% of hemoglobin is abnormal.
 No symptoms
Diagnosis
 Can be diagnose prenatally by chorionic villi
sampling or from cord blood during
amniocentesis
 Attacks are diagnosed clinically
 Abnormal hemoglobin forms are detected on
hemoglobin electrophoresis, a form of gel
electrophoresis on which the various types of
hemoglobin move at varying speed
 sickledex
Characteristics of Sickled Cells
Normal RBC Sickled
Cells
120-day life span 30- to 40- day
life span

Hgb has normal Hb has

O2 carrying decreased O2
capacity carrying
capacity
12 to 14 g/ml of 6 to 9 g/ml of Hb
Hb

RBC destroyed at RBCs destroyed at

accelerated rate
CRISES
1. Vaso-occlusive— “painful episode”
2. Acute splenic sequestration-- pooling of blood
3. Aplastic– diminished RBC production
4. Hyperhemolytic– accelerated rate of RBC
destruction
5. Cerebrovascular accident– blockage of major
blood vessels
6. Acute Chest syndrome– similar to pneumonia
7. Infection
 Change in one base-pair in DNA molecule

Valine produced instead of glutamic

acid at position, 6 in β-chain

Desctruction Abnormal hemoglobin molecule Concentration

of many of sickle-
sickle cells shaped cells in
Sickling of RBC the spleen

Clumping of sickle shaped cells

Enlargement
Anemia interferes with circulation
of spleen

Proliferation Impaired blood supply to

of bone Enlargement various organs Fibrosis of
marrow of heart spleen

Weakness Slowed
and physical Damage Damge Damage Brain Damage
lassitude development to heart to lungs to damage to abd Kidney
muscle muscles organs damage
Impaired and
mental joints
function
Heart pneumonia paralysis Kidney
Abd
Failure Rheumatism pain failure

DEATH
NURSING PROBLEMS

 Impaired
gas  Ineffective tissue
exchange perfusion
 Dyspnea  paralysis
 Use of accessory  Tissueinfarction
muscle  Bone pain
 Cyanosis

 Hypoxia

 restlessness
NURSING PROBLEMS
 Acute/ Chronic pain • Delayed growth
 Localized/ and development
generalized joint and/
-Altered physical
or abdominal/ back
pain
growth
 Guarding -Delay and difficulty
 Crying, restlessness performing skills
 Facial grimacing
Sickle cell anemia as an
inflammatory disease

Orah S. Platt
Harvard Medical School, Children’s Hospital,
300 Longwood Avenue, Boston,
Massachusetts 02115, USA.
Sickle cell anemia as an inflammatory
disease
Classical view---“primary genetic defect”:
abnormal Hgb
Holistic view---abnormal hgb interacts with,
damages, and stimulates the vascular
endothelium “irritant”
“. . .reperfusion injury plays a major role
in sickle pathophysiology. . .”
Sickle cell anemia as an inflammatory
disease
 high
base-line leukocyte count
ongoing base-line chronic inflammation

major risk factor for severity in sickle cell

anemia
References
 Andreoli & Bennett etal; Cecil Essential of Medicine, 4th Edition,
1997, WB Saunders Co
 Bullock: Pathophysiolgy: Adaptations and Alterations in Function, 4th
Edition; 1996, Lippincott
 Fauci et al: Harrison’s Principle of Internal Medicine, 17th Edition:
McGraw Hill Companies, Inc
 Mader: Understanding Human Anatomy Physiology, Fifth Edition,
The McGraw−Hill Companies, 2004
 Marieb: Essentials of Human Anatomy and Physiology, 6th Edition,
2002, Pearson Education Asia Pte, Ltd
 McPhee at al: Pathophysiology: An Introduction to Clinical Medicine,
2nd Edition, 1997, Prentice Hall, ltd
 Rifknd et al: Fundamentals of Hematology, 2nd Edition; 1980; Year
Book Medical Publishers, Inc
 Straight A’s in Pathophysiology: A Review Series; Lippincott
Williams & Wilkins