Hemolytic Anemia

Type Of Anemia
HEMOLYTIC ANEMIA:-
 Hemolytic anemia caused by increased destruction
of red cells or due to a short life span of red cells.
Pathogenesis:-
 The normal life span of the red cell is about 120
days.
 When there is hemolysis of bonemarrow compensates
by increasing erythropoiesis by 6 to 8 times the
normal number of RBC produced.
 Anemia results when the bone marrow is no
longer able to compensate the degree of hemolysis.
Symptoms Of Hemolytic Anemia :-
 Paleness of skin
 Confusion
 Tiredness
 Fever
 Weakness
 Dizziness
 Increased heart rate
 Dark urine
CLASSIFICATION OF HEMOLYTIC ANEMIA On The Basis Of
Causes Of Hemolytic Anemia :-
Based on the site of red cell destruction, hemolytic
anemia is classified as:
Intravascular:destruction is within the blood circulation
Extravascularr: destruction is within tissue
macrophages.
The macrophages in the spleen, liver bone marrow and
lymph nodes phagocytose and destroy red cells.
Defects inside the RBC Or Intracorpuscular defects:-
 Red cell membrane defects - Hereditary spherocytosis,
elliptocytosis.
 Abnormalities of hemoglobin synthesis
1 . Decreasedd globin synthesis – quantitative defect eg.
Thalassemias
2 . Abnormal globin synthesis – qualitative defect eg.
Sickle cell anemia
 Abnormalities of red cell enzymes – eg. G6PD
deficiency.
Defects outside the RBC (Extracorpuscular defects):-
The red cells are normal but the life span is shortened
because of external factors.
 Immune hemolytic anemia- alloimmune, autoimmune,
drug induced
 Parasites – eg. Malaria
 Bacterial – eg. Clostridia
 Venoms – eg. Snake venoms.
 Drug induced
 Burns
 hemolytic uremic syndrome:- In this condition, small
blood vessels of kidney damaged, due to which clotting
occur and filtration is interrupted in the, which leads to
kidney failure.
 March hemoglobinuria :- Large amount of hemoglobin
present in the urine because of large hemolysis. The
damage is due to repetitive prolonged marching and marathon
running.
 Prosthetic heart valves :- Artificial heart valve.
 Red cell fragmentation intravascular coagulation :-
Clotting occurs due to damaged blood vessels, due to
which Hemolysis occurred.
LABORATORY FEATURES Of HEMOLYTIC ANEMIA:-
 Hemoglobin, PCV and RBC count are reduced.
 Reticulocyte count is increased.
 Red cell life span is decreased.
Peripheral blood smear:-
 variable anemia with anisocytosis,poikilocytosis,
polychromasia, fragmented red cells, nucleated RBC and
basophilic stippling.
 sickle cells, spherocytes or target cells may be present.
 Bone marrow examination :-
 has limited role in the diagnosis of hemolytic
 anemia. The bone marrow shows cellular marrow
particles and markedly
 hypercellular smears. There is marked erythroid
hyperplasia with
 normoblastic maturation resulting in reduction of
myeloid to erythroid
 ratio. Myeloid maturation and thrombopoiesis are
normal.
BIOCHEMICAL CHANGES DURING HAEMOLYSIS
 When the red cells are destroyed, the hemoglobin is
broken down to globin and heme.
 Globin chains are broken down to amino acids which are
re-used for protein synthesis.
 The heme ring is opened up and the iron is released,
bound to transferrin,recirculated and used for new RBC
formation.
 The protoporphyrin ring is opened up and during this
process carbonmonoxide is released and excreted
through expired air.
 Biliverdin is formed from the protoporphyrin. Biliverdin is
converted to un-conjugated (indirect) free bilirubin by
biliverdin reductase.
 The unconjugated bilirubin is bound loosely to serum
albumin and is conjugated to bilirubin glucuronide (direct or
conjugated bilirubin) in the liver.
 The conjugated bilirubin is excreted in bile giving it a yellow
green colour
 Most of the bile secreted in the small intestine is reabsorbed
and transported back to the liver. This is called the
enterohepatic circulation of bilirubin.
 In the intestine the remaining bilirubin is converted to
stercobilinogen
 Some of the stercobilinogen is reabsorbed and excreted as
urobilinogen in urine.
 Thus in hemolytic anemia, the serum total bilirubin is
mildly increased,most of it is indirect bilirubin, urine
urobilinogen and fecal stercobilinogen are increased
and there is increased storage iron.
 When red cells get destroyed in the blood stream
releasing hemoglobin into plasma it is called
intravascular hemolysis.
 The hemoglobin binds to a plasma protein called
haptoglobin and the Hb-Haptoglobin complex is taken
up by Tissue macrophages.
 The hemoglobin is then degraded to biliverdin. When all
The haptoglobin is saturated the free hemoglobin is
increased resulting in Hemoglobinemia and is excreted
by the kidney as hemoglobinuria.
 Some of this hemoglobin in the glomerular filtrate is
reabsorbed by the tubular epithelium And stored as
hemosiderin.
 Demonstration of hemosiderin in
urine(hemosiderinuria) indicates recent intravascular
hemolysis.