Lesson 1

Question 1

 Life saving substance of every red blood cell
 The oxygen-carrying component of the RBC
 Each RBC is nothing more than a fluid-filled
sac with the fluid being hemoglobin
 Also spelled as haemoglobin, iron containing
protein in the blood pof many animals- in the
RBC (erythrocytes) of vertebrates- that
transport oxygen to the tissues.
 Hemoglobin forms an unstable reversible bond
with oxygen
 In the oxygenated state, it is called
oxyhemoglobin and is bright red; in the
reduced state, it is purplish blue
 When red cell die, hemoglobin is broken up:
iron is salvaged, transported to the bone
marrow by proteins called transferrins, and
used again in the production of new RBC
 The remainder of the hemoglobin forms the
basis of bilirubin, a chemical that is excreted
into the bile and gives the feces their
characteristics yellow-brown colour.

Answer 1

HEMOGLOBIN

Question 2

• this portions involves the four iron
  atoms in the ferrous state (Fe2+)
• iron in the ferric state (Fe3+) cannot
  bind oxygen
• surrounded by protophorphyrin IX or
  the phorphyrin ring, a structure formed in
  the nucleated RBC
  
  • Protophorphyrin is the final product in
    the synthesis of the heme molecule.

Answer 2

Heme

Question 3

-this structure consists of amino acids
linked together to form a
polypeptide chain, a bracelet of
amino acids.
-the most predominant chains for
adult hemoglobins are the alpha
and beta chains
- Alpha chains have 141 amino acids in
a unique arrangement, and beta
chains have 146 amino acids in a
unique arrangement.

Answer 3

Globin

Question 4

 Oxygen delivery
 Capable of pulling CO2 away from the tissues
and keeping the blood in a balanced pH.

Answer 4

Function of Globin

Question 5

 Conjugated protein that serves as the vehicle
for the transportation of oxygen and carbon
dioxide.
 When fully saturated, each gram of
hemoglobin holds 1.34 ml of oxygen
 The red cell mass of adult contains
approximately 600g of hemoglobin, capable of
carrying 800 ml of oxygen.

Answer 5

HEMOGLOBIN STRUCTURE

Question 6

1. Hb A is the major (96-98%) normal adult
   hemoglobin.
2. Hb F is the major hemoglobin of the fetus
   and the newborn infant. Only traces of Hb
   F (<1.0%) are found in adults.
3. Hb A2 account for 1.5% to 3.5% of normal
   adult hemoglobin.

Answer 6

TYPES OF HEMOGLOBIN

Question 7

is the major (96-98%) normal adult
hemoglobin.

Answer 7

Hb A

Question 8

is the major hemoglobin of the fetus
and the newborn infant. Only traces of Hb
F (<1.0%) are found in adults.

Answer 8

Hb F

Question 9

account for 1.5% to 3.5% of normal
adult hemoglobin.

Answer 9

Hb A2

Question 10

 Increasing the amount of any of these
abnormal hemoglobins in the bloodstream can
be potentially fatal.
 The production of abnormal hemoglobins
results from accidental or purposeful ingestion
of absorption of substances, drugs that are
harmful.
 Abnormal hemoglobins sometimes are
produced as a result of inherited defects.

Answer 10

ABNORMAL HEMOGLOBINS

Question 11

• iron has been oxidized to the Ferric
  state (Fe3+)
  
  • builds up in the circulation, if the
    level is greater than 10%, individualsappear
    cyanotic (blue color), especially fingers
    and lips
  • aniline drugs and anti- malarial
    treatments may induce a
    methemoglobinemia.

Answer 11

Methemoglobin

Question 12

• hemoglobin with an affinity to
  carbon monoxide (CO) that is 200 times
  greater than its affinity for oxygen
  
  • therefore no oxygen is delivered
    to the tissues
  • levels increases in smokers
    and certain industrial workers
  • carbon monoxide poisoning.

Answer 12

Carboxyhemoglobin

Question 13

agents such as sulfonamides or sulfa-
containing drugs.
- the affinity of sulfhemoglobin for
oxygen is 100 times lower than that of
normal hemoglobin.
- it may be toxic at a very low
level

Answer 13

Sulfhemoglobin

Question 14

• medical terms for a group of blood
  disorders and diseases that affect RBC
  
  • genetic defect that results in
    abnormal structure in one of the
    globin chains of hemoglobin
    molecule.

Answer 14

Hemoglobinopathies

Question 15

 Common hemoglobinopathies is sickle cell and
thalassemia
 May cause anemia inherited
 Hemoglobin produced by genes that control
the expression of hemoglobin protein
 Abnormal hemoglobin appear in one of the
three basic circumstances:

Answer 15

Hemoglobinopathies

Question 16

Abnormal hemoglobin appear in one of the
three basic circumstances:

Answer 16

Structural defects in the hemoglobin
molecule

Diminished production of one of
the two subunits of the
hemoglobin molecule

Abnormal associations of otherwise
normal subunits

Question 17

• alterations in the gene for one of
  the two hemoglobin subunits
  chains, alpha or beta, called
  mutations
  
  • alteration of a single amino
    acids disturbs the behavior of the
    hemoglobin molecule, and
    produces disease
    state.
  • sickle hemoglobin exemplifies
    this phenomenon

Answer 17

Structural defects in the hemoglobin
molecule

Question 18

-Equal numbers of hemoglobin
alpha and beta chains are
necessary for normal function
-Hemoglobin chain imbalance
damages and destroys red
cells thereby producing anemia.

Answer 18

Diminished production of one of
the two subunits of the
hemoglobin molecule

Question 19

-Beta globin subunits begin to
associate into groups of four
due to paucity of potential alpha
chain partners.
-These tetramers of beta globin
subunits are functionally
inactive and do not
transport oxygen.

Answer 19

Abnormal associations of otherwise
normal subunits

Question 20

• A predominant hemoglobin in people
  with sickle cell disease
• alpha chain is normal
  -disease producing mutation exist in beta
  chain
• if one single mutant gene and one
  normal beta gene,sickle cell trait is
  benign

Answer 20

Hemoglobin S

Question 21

• results from a mutation in the beta globin
  gene
  -Hemoglobin C disease is relatively
  benign, producing mild hemolytic
  anemia and splenomegaly.

Answer 21

Hemoglobin C

Question 22

• results from the mutation in the
  hemoglobin beta chain manifest mild
  hemolytic anemia and mild
  splenomegaly.

Answer 22

Hemoglobin E

Question 23

• tetramer composed of four beta
  globin chains
  -occurs only with extreme limitation of
  alpha chain availability

Answer 23

Hemoglobin H

Question 24

• variant in which the mutation in the
  alpha globin gene produces an alpha
  globin chain that is abnormally long.

Answer 24

Hemoglobin Constant Spring

Question 25

• develops in fetuses with four gene
  deletion alpha thalassemia

Answer 25

Hemoglobin Barts

Question 26

• occurs when someone inherits two
  different variant genes from the alpha globin gene
  cluster or two different variants genes from the beta
  globin gene cluster

Answer 26

COMPOUND HETEROZYGOUS CONDITIONS

Question 27

• patients inherit a gene for hemoglobin S
  from one parent, and a gene for
  hemoglobin C from the other.
  -Hemoglobin C interacts with
  hemoglobin S to produce with some
  abnormalities seen in patients with
  sickle cell disease.
• Hemoglobin SC is more severe than
  homozygous hemoglobin C disease
• Hemoglobin SC is milder than those
  with sickle cell disease

Answer 27

Hemoglobin SC disease

Question 28

• patient has inherited a gene for
  hemoglobin S from one parent and a
  gene for beta-thalassemia from the
  other.
• its severity is determined to a large
  extent by the quantity of normal
  hemoglobin produced by beta-
  thalassemia gene.

Answer 28

Sickle/beta-thalassemia

Question 29

-this produces a condition more severe
than is seen with either hemoglobin E
trait or beta-thalassemia trait.

Answer 29

Hemoglobin E/beta -thalassemia

Question 30

• affect alpha globin gene cluster

Answer 30

Alpha thalassemia/Hemoglobin constant
spring

Question 31

• a blood disorder in which the normal
  hemoglobin protein is produced in
  lower amount as usual.
• Inherited
• Occurs when there’s an
  abnormality or mutation in one of the genes involved in hemoglobin
  production

Answer 31

THALASSEMIA

Question 32

• subtypes: major
  and intermedia
• occurs when a body
  cannot produce beta globulin

a. Thalassemia major - most severe form of
beta thalassemia
- develops when beta globin genes
are missing
- symptoms generally appear
before a child’s second birthday
- life threatening
- severe that usually requires blood
transfusion

b. Thalassemia intermedia - less severe for,
- develop because of alterations
in both beta globin genes
- may not need blood
transfusions

Answer 32

Beta thalassemia

Question 33

• occurs when the body
  can’t make alpha globin
• fewer red cells and less hemoglobin than
  it should

Types:
 Hemoglobin H
 Hydrops fetalis

Answer 33

Alpha Thalassemia

Question 34

• less serious form of
  disorder
  -If one of the parents is a carrier of
  thalassemia, a child may develop this
  type of thalassemia, but will only be a
  carrier, that may not have symptoms.
• if both parents are carrier, a child may
  have a greater chance of inheriting a
  more serious form of the disease

Answer 34

Thalassemia minor

Question 35

 If your doctor is trying to diagnose thalassemia,
they’lllikely take a blood sample. They’ll send
this sample to a lab to be tested for anemia
and abnormal hemoglobin. Alab technician will
also look at the blood under a microscope to
see if the red blood cells are oddly shaped.
 Abnormally shaped red blood cells are a sign
of thalassemia. The lab technician may also
perform a test known as hemoglobin
electrophoresis. This test separatesout the
different molecules in the red blood
cells,allowing them to identify the abnormal
type.

Answer 35

Diagnosis of Thalassemia

Question 36

Treatment Options

Answer 36

1. Blood transfusion - doctors may instruct not
   to take vitamins or supplements containing
   iron need a chelation therapy.
2. Bone marrow transplant
3. Medications and supplements

Question 37

 The measurement of hemoglobin is one of
several test used to diagnose and follow the
treatment of anemia. The normal range for the
hemoglobin will vary with the age and sex of
the individual.
 At birth, the hemoglobin concentration is
normally in the range of 15 to 20g/dl.
 At 2 months, values decreases to about 9 to
14 g/dl
 At 10 years of gae, the normal hemoglobin is
between 12 to 15 g/dl.
 Normal adult values range from 12-16 g/dl in
women and 13-18 g/dl for men.
 Hemoglobin is measured as oxyhemoglobin.
 Some methods measure oxyhemoglobin
indirectly by converting it to compounds like
acid hematin and cyanmethemoglobin, alkali
hematin and carboxyhemoglobin. Other
indirect methods determine hemoglobin levels
by detecting the concentrations of oxygen and
iron carried by hemoglobin molecule as well as
by the blood’s specific gravity.

Answer 37

HEMOGLOBIN DETERMINATION

Question 38

Principle: Conversion of hemoglobin to
acid hematin by using 0.1N HCI.
The yellowish brown solution is
compared to the color standard inthe
comparator block.

Answer 38

Acid Hematin Method

Question 39

Principle: Drabkin’s reagent is used to
measure hemoglobin through
cyanmethemoglobin method. It consist of
sodium bicarbonate, potassiumcyanide
and potassium ferricyanide.

 Cyanmethemoglobin method is the best
method of manual determination.

Answer 39

Cyanmethemoglobin/HemiglobinCyanide Method

Question 40

1. Its pigment is stable even in diluted solution.
2. The method measures all hemoglobin
   except sulfhemoglobin.
3. Certified Cyanmethemoglobin standard is
   commercially available.
4. The spectral curve of cyanmethemoglobin
   allows the use of different types of
   spectrophotometers.

Answer 40

Advantages Of Cyanmethemoglobin

Question 41

Other Methods Of Hemoglobin Determination

Answer 41

I. Colorimetric Method
1. Talquist scale
2. Dare Hemoglobinometer
Il. Indirect Method
1. Oxyhemoglobin
2. Carboxyhemoglobin
Ill. Gasometric Method
1. Van Slyke Oxygen Capacity
IV. Hemoglobin electrophoresis