Anemia- a decrease in total amount of RBC or hemoglobin in blood
can be caused due to blood loss, iron or vitanminB12 deficiency
thalassemia
hemolysis-destruction of red blood cell
hemolytic anemia- a disorder where RBBC are destroyed faster than they are made.
1st year MBBS
3. Definition
Hemolytic anemia is a disorder in which red blood cells are destroyed faster than
they can be made. The destruction of red blood cells is called hemolysis.
Defects causing premature red cell destruction may be divided into two categori
es- Intracorpuscular and Extracorpuscular.
5. INTRACORPUSCULAR
DEFECTS
EXTRACORPUSCULAR DEFECTS
HEREDITARY •Membrane defects-Hereditary spherocytosis, h
ereditary elliptocytosis
•Hemoglobin defects-
I. Haemoglobinopathies: Sickle cell anemia,
abnormal hemoglobins (such as HbC,
HbE,HbD,etc.) and unstable hemoglobin di
sease.
II. Thalassemia: Thalassemia major and mino
r.
•Enzyme defects- Deficiency of pyruvate kinase
and glucose-6-phosphate dehyrogenase
Familial hemolytic uremic
syndrome
ACQUIRED Paroxysmal nocturnal
hemoglobinuria
I. Immune mechanisms: Warm and cold an
tibody autoimmune hemolytic anemia, h
emolytic disease of newborn,
incompatible blood transfusion,
I. drug induced anemia
II. Iso immune hemolytic anemia
III. Infections - malaria
IV. Burn
V. Snake bite
6.
7. Hereditary Spherocytosis
• An autosomal disorder which affects males and females equally
• Type of hemolytic anemia- increased defect in red cell membrane due to loss of spectrin which
results spherocytic shape of the cell.
• Generally, this type of anemia is normochromic and normocyctic
• Surface area: volume ratio and cells are more rigid
• More prone to rupture
• Features: anemia, jaundice, enlarged spleen and may present with gallstone
• Increased osmotic fragility, hyperbilirubinemia and reticulocytosis
• Short life expectancy
8. Sickle cell Anemia
• It is an inherited red blood cell disorder which is caused
by abnormal hemoglobin HbS.
• HbS is the hemoglobin in which glutamic acid is replaced by valine in
the 6th position of beta chain.
• Following deoxygenation, the mutated hemoglobin (HbS) molecules
polymerize to form bundles.
• The polymer bundles result in erythrocyte sickling, which in turn
results in impaired rheology of the blood and aggregation of sickle
erythrocytes with neutrophils, platelets and endothelial cells to prom
ote stasis of blood flow.
• Vaso-occlusion promotes ischemia
• Hemoglobin (Hb) polymer bundles also promote hemolysis.
9. Thalassemia
Thalassemia is an inherited blood disorder characterized by reduction in the rate of one or more types of
hemoglobin polypeptide chain(α or β).
Also leads in reduction of the ability of the red cells carrying oxygen.
It is categorized into two major classes: α-thalassemia and β-thalassemia
Alpha-thalassemia Beta-thalassemia
•Arises due to insufficient synthesis of alpha-hemoglobin
chains and an excess of beta chains.
• It has less impact than beta thalassemia
• Occurs due to insufficient synthesis of beta-hemoglobin
chains and an excess of alpha chains.
• It will produces more serious illness than alpha thalasse
mia
10.
11. • A genetic disorder that occurs almost exclusively in males
• In affected individuals, a defect in an enzyme called glucose-6-
phosphate dehydrogenase cause hemolysis.
• It renders RBCs susceptible to oxidative stress, which shortens RBC
survival.
• Hemolysis occurs following an oxidative challenge, commonly after
fever, acute viral or bacterial infections, and diabetic Ketoacidosis
• Less commonly, hemolysis occurs after exposure to drugs or to other
substances that produce peroxide and cause oxidation of hemoglobin
and RBC membranes.
• These drugs and substances include primaquine, salicylates, sulfonam
ides, nitrofurans, phenacetin, naphthalene, some vitamin K derivative
s, dapsone, phenazopyridine, nalidixic acid, methylene blue and fava
beans.
Glucose-6-phosphate dehydrogenase deficiency
12.
13. IMMUNOHEMOLYTIC ANEMIA
• Immunohemolytic anemias are a group of anemias occurring due to antibody production by the
body against its own red cells.
• It is induced by one of the following three types of antibodies:
1.Autoimmune hemolytic anemia
a. Warm antibody AIHA
b. Cold antibody AIHA
2.Drug induced immunohemolytic anemia
3.Isoimmune hemolytic anemia
14. Symptoms
•Abnormal paleness or lack of color of the skin
•Yellowish skin, eyes, and mouth (jaundice)
•Dark-colored urine
•Fever
•Weakness
•Dizziness
•Confusion
•Can’t handle physical activity
15. Signs
Signs of anemia:
•Pale appearance of skin
•Tachycardia, tachypnoea and hypotension
•Mild jaundice occur due to hemolysis
•Splenomegaly occurs in spherocytosis
•Leg ulcers occurs in sickle cell anemia
16. Investigation
Test for hemolysis:
• Complete hemogram
• Serum bilirubin level
Test for increased red cell production:
• Pheripheral smear
• Reticulocyte count
17. Investigation
Test of damage to red cell:
Spherocytosis: peripheral blood smear
Thalassemia are diagnosed by:
•Hemoglobin electrophoresis
•Demonstration of Hb-H chains( in absence of sufficient
alpha chains, excess of beta or gamma chains aggregate t
o form Hb-H)
•Study of rate of globin synthesis
18. Investigation
Test of damage to red cell:
Diagnosis of Sickle cell anemia is done by:
•sickle test,
•Hemoglobin solubility test
•Hemoglobin electrophoresis
Diagnosis of Glucose-6-Phosphate Dehydrogenase
deficiency:
•a complete blood count,
• serum hemoglobin test,
•a reticulocyte count
•Peripheral blood smear
Diagnosis of autoimmune hemolytic anemia:
• Coomb’s test.