CHAPTER TWO

REVIEW OF RELATED ANATOMY

REVIEW OF RED BLOOD CELLS (ERYTHROCYTES)
Red blood cells are by the most abundant types of blood cell 99% of all blood cells
are erythrocytes. They are biconcave discs with no nucleus, and their diameter is
about 7um. Their main function is in gas transport, mainly of oxygen, but they also
carry source carbondioxide. Their characteristics shape is suited to their purpose,
the biconcavity increases their surface are for gas exchange, and exit of gases. The
cells are flexible so they squeeze through narrow capillaries, and contain no
intracellular organelles, leaving more room for haemoglobin, the large pigmented
protein responsible for gas transport.

Adopted from:
Normal Red Blood Cells
Waugh, A. & Grant, A. (2010). "Ross and Wilson
Anatomy and Physiology in health and illness"
Edinburgh: Churchill Livingstone. Page 64

LIFE SPAN OF ERYTHROCYTE

Erythrocyte have no nucleus, cannot divide and so need to be continually replaced
by new cells from the red bone marrow, which is present in the ends of long bones,
flat bones and irregular bones. They pass through several stages of development
before entering the blood. Their life span in the circulation is about 120 days.
DEVELOPMENT / FORMATION OF ERYTHROCYTE
The process of development of red blood cells from stem cells takes about 7 days
and is called erythropoiesis. The immature cells are released into the blood stream
as reticulocytes and mature into erythrocytes one a day or two within the
circulation. During this time, they lose their nucleus and therefore become
incapable of division. It starts from the proerythroblast. The erythroblast
accumulates hemoglobin and iron and gradually loses its nucleus. At this stage, the
cell is known as reticulocyte and it loses the dark stained material within the cell,
thus becoming an erythrocyte and released into the blood stream.
On increased demand for red blood cell, reticulocytes are released permanently
into the circulation and more nucleated red blood cells are seen within circulation,
then over a day or two, they mature into an erythrocyte.
The production of erythroblasts from the stem cell is stimulated by a hormone
produced by the kidney called erythropoietin. When there is hypoxia i.e. deficient
oxygen supply to cells, the kidney detects it and stimulates erythropoietin
production which stimulates the bone marrow to produces erythrocyte.

For the formation of the red blood cell, vitamin B12, and folic acid are essential.
They are absorbed in the small intestine, although vitamin B12 must be bound to
intrinsic factor to allow absorption to take place.
Pluripotent stem cell
Proerythroblast
Erythroblast
Reticulocyte
Erythrocyte
Haemopoeisis: Stages in the development of red blood cell.
Adopted from:
Waugh, A. & Grant, A. (2010). "Ross and Wilson
Anatomy and Physiology in health and illness"
Edinburgh: Churchill Livingstone. Page 64

HAEMOGLOBIN
Haemoglobin is a large, complex protein containing a globular protein (globulin)
and a pigmented iron containing a complex called ham. Each haemoglobin
molecule contains four globins chains and four haem unit, each with one atom of
iron. As each atom of iron can combine with oxygen molecule, this means that a
single heamoglobin molecule can carry upon to four molecules of oxygen. An
average red blood cell carries about 280 million hemoglobin molecules, giving
each cell a theoretical oxygen-carrying capacity of over a billion oxygen
molecules.
Iron is carried in the blood stream bound to its transport protein, transferin and
stored in the liver. Normal red blood cell production requires a steady supply of
iron. Absorption of iron from the alimentary canal is very slow even if the diet is
rich in iron, meaning that iron deficiency can readily occur if it loses exceed
intake.
DESTRUCTION OF ERTHROCYTE
The life span of erythrocytes is about 120 days and their breakdown, or haemolysis
is carried out by phagocytic reticuloendothelial cells. These cells are found in
many tissues but the main sites of haemolysis are the spleen, bone marrow and
liver. As erythrocyte age, their cell membrane becomes more fragile and so more
susceptible to haemolysis. Iron released by haemolysis is retained in the body and
revised in bone marrow to form haemoglobin. Bilverdin is formed from the haem
part of haemoglobin. It is almost completely reduced to the yellow pigment
bilirubin, before being bound to plasma globulin and transported to the liver. In the
liver it is changed from a fat-soluble to water-soluble form to be excreted as a
constituent of bile.

FUNCTIONS OF ERYTHROCYTE
1. Transportation of haemoglobin which in turn carries oxygen from lung to
tissue.
2. Transport of carbon dioxide from tissue to lungs.
3. Production of haemoglobin.
Normal values of erythrocyte and haemoglobin
Measures
Normal Values
Erythrocyte count- number of
Male: 4.5 x 10'2/L to 6.5 x 10'2/L
Female: 3.8 x 10'2/L to 5.8 x 1012/L
erythrocytes per litre, or cubic milliliter
(mm') of blood
Packed cell volume (PCV, haematocrit)-
the volume of red cells in 1 litre or mm
3
blood.
0.40 - 0.55 L/L
Haemoglobin- the weight of
Male: 13 - 18 g/100ml
haemoglobin in whole blood measured in Female: 11.5 - 16.5 g/100ml
grams/100ml blood

REVIEW OF DISEASE CONDITION: HAEMOLYTIC ANAEMIA

Definition of Haemolytic Anaemia
Haemolytic Anemia is when circulating red cells and destroyed or are removed
prematurely from the blood because the cells are abnormal or the spleen is
overactive.
CAUSES OF HAEMOLYTIC ANAEMIA
Haemolytic anemia is caused by some certain factors, diseases and even
medications. The following are some underlying cause of extrinsic haemolytic
anaemia;
Hepatitis spleen
Hepatitis
Typhoid fever
E. coli toxin
Leukemia
Lymphoma
Tumours
The intrinsic cause develops when the red cells produced by your body don't
function properly. This condition is inherited such as people with sickle cell
anemia or thalassemia.
Others include; deficiency or red blood cell membrane instability can lead to the
condition, medication such as acetamophen, penicillin, ibuprofen.

CLASSIFICATION OF HEMOLYTIC ANAEMIA

Hemolytic anaemia may be classified in several ways;
Congenital hemolytic anaemia
Haemolytic disease of the newborn
Acquired hemolytic anaemia
Autoimmunity
Congenital Haemolytic Anemia: In this disease, genetic abnormality leads to the
synthesis of abnormal haemoglobin and increased red cell membrane fragility,
reducing their oxygen-carrying capacity and life span. The most common forms
are sickle cell anemia and thalassaemia,
Haemolytic Disease of the Newborn: In this disorder, the method immune system
makes antibody to the baby's red blood cells, causing destruction of fetal
erythrocytes.
Acquired Haemolytic Anaemia: In this context,
"acquired' means haemolytic
anemia in which no familial or racial factors have been identified it is caused by
several things such as;
Some
drugs, especially when taken long term in large discs
e.g.
sulphonamides.
Chemicals encountered in the general or work environment e.g. lead, arsenic
compounds.
Toxins produced by microbes; e.g. streptococcus pyrogenes, clostridium
perfringens.
Autoimmunity: In autoimmunity, individuals make antibodies to their own red
cell antigen, causing haemolysis.

CLINICAL MANIFESTATION
Signs and symptoms of hemolytic anemia are disease and are due to anemia, the
extent of compensation, previous treatment and the underlying disorder. Patients
with minimal or long-standing hemolytic anaemia may be asymptomatic, and
haemolysis is often found incidentally during routine laboratory testing. Clinical
manifestation may include the following;
Intravascular hemolysis, iron deficiency due to chronic haemoglobinuria can
exacerbate anemia and weakness.
Tachycardia, dyspnea, angina, and weakness occur in patients with severe
anemia, as cardiac function is sensitive to anoxia.
Persistent hemolysis may result in the development of bilirubin gallstones,
these patients may present with abdominal pain.
Bronze skin color and diabetes occur in haematosiderosis, iron overload
may occur in patient who have received multiple transfusions or those who
have been administered iron therapy erroneously.
In addition to hemolysis, patients with thrombotic thrombocytopenic purpura
(TTP) may
expensive
fever, neurologic
signs,
renal
failure
and
thrombocytopenia.

ASSESSMENT OF DIAGNOSTIC FINDINGS

A number of hematologic studies are performed to determine the type and
cause of anaemia.
• Blood studies: in an initial
evaluation, the hemoglobin hematocrit,
reticulocyte count and the BC includes particularly the mean corpuscular
volume and red cell distribution width are taken to assess for presence of
anaemia.
• Iron studies: serum iron level total iron binding capacity percent saturation
and ferritin, as well as serum vitamin Biz and folate level are all useful in
diagnosing anaemia
CBC value: the remaining CBC values are useful in determining whether the
anemia is an isolated problem or part of another hematologic condition.

PATHOPHYSIOLOGY
Hemolytic anemia results from either intravascular or extravascular red blood cell
destruction. Extravascular hemolysis results from accelerated red cell destruction
by cells of the reticuloendothelial system, due to immune targeting by antibodies,
as occurs in a warm autoimmune hemolytic anemia. Intravascular hemolysis
results from red cell destruction within the vasculature, due to complement-
mediated lysis or directed red cell trauma from a prosthetic heat halve or
microangiopathic process.
Autoimmune haemolysis is most often due to extravascular destruction of red cells
coated with an autoantibody. Because sections of the BC membrane are removed
by the reticuloendothelial cells, RBCs with a smaller surface area appear as
spherocytes on the peripheral smear. In contrast, traumatic disruption of red cells
by microangipathic processes results in the production of red cells fragment or
schistocytes on the smear. This results in the release of RBC, LDH and Hb into the
circulation, which can result in haemoglobinuria. Free haemoglobin bound by
haptoglobin that is observed in many cases of hemolytic anemia. Hereditary
causes of hemolytic anemia, including membrane defects, enzyme mutations, and
hemoglobinopathies, generally result in anemia due to significantly shortened
BC life span and removal of senescent red cells by the spleen.

MEDICAL MANAGEMENT OF ANAEMIA

Management of haemolytic anemia is directed towards correcting or controlling
the cause of anaemia.
•
Nutritional supplement;
Isuse
of nutritional supplement should
be
appropriately taught to the patient and family because nutritious foods will
help to build up body tissues.
Blood transfusion; patient with acute blood loss or severe haemolysis may
have decreased tissue perfusion from decreased blood volume or reduced
circulating erythrocytes, so transfusion of blood would be necessary
Intravenous fluids; intravenous fluids replaces the lost volume of blood or
electrolyte to restore then to normal level.
Medications like fersolate, vitamin B-complex, vitamin c which helps in the
formation of haemoglobin during erythropoiesis and thereby helping to
prevent and treat iron deficiency anaemia.

NURSING MANAGEMENT
The management of haemolytic anemia by the nurse should be accurate and
appropriate so that objectives and goals would be achieved.
Admission: Patient was admitted on a well made bed and a well ventilated ward
- patient was admitted near a patient with the same condition to allay anxiety of the
patient and family members.
Position: Patient was placed on a comfortable position.
Psychological Care: Patient and relatives are reassured and the nature of illness
explained to them. A good nurse patient therapeutic relationship should be created
to allay anxiety and encourage to verbalize fear.
Observation: Patient is monitored closely, vital signs (temperature, pulse,
respiration and blood pressure) were monitored at regular interval during blood
transfusion and during her stay in the hospital. Urine was also observed in case
there is presence of blood and keep strict intake and output chart.
Nutrition: Adequate nutrition were given to the patient.
Investigation: Ordered investigations were carried out, like full blood count,
malaria parasite test, hepatitis B, erythrocyte sedimentation rate. All these were
carried out to detect any abnormality and also the results attached to patient's
folder.
Rest: Patient should maintain adequate rest and sleep to conserve energy.
General Care: Patient should take her bathe every morning and also oral toileting.

Discharge: Patient is advised to maintain a good hygiene, eat adequate diet, take
her drugs as prescribed, come to cheek up when due or if any problem arises; avoid
stressful conditions.
PREVENTION
• To prevent haemolytic anemia, life style modification must be made.
• Diet rich in iron; ingestion of iron-rich food could help prevent haemolytic
anaemia because it adds to the haemoglobin in the body.
• Iron supplement; iron supplement can also be taken to increase the
hemoglobin level in the body.
PROGNOSIS
Haemolytic anemia generally has a very good prognosis and it maybe curable in
many instances. The overall prognosis depends on the underlying cause of
anemia, its severity and the overall health of the patient.
COMPLICATIONS
General complication of severe hemolytic anaemia include;
Heart failure.
Paresthesias.
Delirum.