Normal shape of RBC- biconcave
Normal life span of RBC-120 day
Hemolytic anemia is a form of anemia due to
haemolysis ,the abnormal premature breakdown of
red blood cells (RBCs)either in the blood vessel
(intravascular haemolysis)or elsewhere in the human
body(extravascular ) and bone marrow is not
compensating for red blood cell loss.
Compensatory action of bone
In response to haemolytic anaemia –dec haemoglobin
Oxygen supply to kidney is dec,
Inc erythropoiten from peritubular capillaries around
Erythropoiten prevents apoptosis of erthroblast
.causes erythroid hyperplasia
Bone marrow can inc prod.of RBC 8 to 10 times.
(compensated haemolytic disease)
evidence of haemolyis Jaundice due to unconjugated hyperbilirubinemia.
jaundice is mild to moderate.
lemon yellow color of skin(Pale color of aneamia and
yellow color of jaundice )
Dark color stool
Inc urobilinogen in urine(acholuric jaundice)
LDH level are raised
Haptoglobin (macrophages destroy haptoglobin and
Slightly less haptoglobin in extravascular
Very less haptoglobin in intravascular.
PERIPHERAL BLOOD FILM
evidence of haemolysis
Normal reticulocyte count in blood is 1 to 2%
In haemolysis reticulocyte count is upto 20%
MCV-Increses(105fl)normal is 80 to 100fl
Polychromasia(multiple color and shade)
Hypercelluar bone marrow
dec haptoglobin or absent haptoglobin
Tetrameric to dimer structure of haemoglobin --
endocytosis by PCT cells ---release of iron-- Acute tubular
necrosis –renal failure
FEATURES OF EXTRAVASCULAR
RBC SHAPE –SPHERICAL
RBC COLOR- DARK RED WITH LOSS OF CENTRAL
ACUTE OR CHRONIC intravascular
Deposition of bilirubin in gall bladder—gall stones.
Haemosiderinurea—due to chronic haemolysis haemosiderin
is formed in PCT.Stained by persian blue.
Intrinsic and extrinsic causes
INTRINSIC CAUSES –cause is related to RBC itself.
EXTRINSIC CAUSES-where factors external to the
Mutation of myeloid cells
On x chromosomes on short arm
PIG-A gene-GPI PHOSPHOLIPIDS on cell membrane which is
attached to PIG tailed protein(CD55,CD59,C-8 BINDER)
Haemolysis of RBC,WBC and platelets.
ASSOSIATION WITH APLASTIC ANAEMIA
AUTOREACTIVE REACTIVE T CELLS DESTROYS THOSE
MYELOID CELL WHICH ARE LOADED WITH NORMAL GPI
PROTEIN WHICH WILL CAUSE CELLS DEFICIENT IN GPI
WILL PROLIFERATE.THESE PATHOLOGICAL CELLS WILL
In severe case pancytopenia
Haemolysis occur all the time
During sleep occurs more
Breathing become slow –inc in co2---produce acidic enviornment-alternate
Thrombi formation(hepatic vein,cerebral vein,deep vein of legs)
Free Hb combine with NO
Smooth muscle fail to relax—erectile dysfuntion,odynophagia
LAP score dec
SUCROSE HAEMOLYSIS TEST
MONOCLONAL ANTIBODIES—BINDS WITH C5 PREVENTS
BREAKDOWN OF C5 TO A AND B COMPONENT.
Haemoglobin synthesis defect
GLOBIN CHAIN SYNTHESIS IS QUANTITATIVELY REDUCED
THALASEMIA(ALPHA OR BETA )
QUALITATIVE DEFECT IN GLOBIN CHAIN
SICKLE CELL ANAEMIA(GENE defect POSITION NO 6 .GLUTAMIC
ACID IS REPLACED BY VALINE)
Immune mediated causes like
ISO ANTIBODY---ABO ,Rh
Warm cold agglutinins cold haemolysins
37 DEGREE CENTIGRADE
DIRECTED AGAINST -Rh antigen
Monomeric cannot agglutinate
Compliment system cannot be activated(RBC has cd59 and
RBC when they pass through spleen converts to
spherocytes.(differentiated by direct coombs test)
losparin,quinidine,alpha methyl dopa)
<37 degree centigrade
Works only when peripheral parts are exposed to cold
Acrocyanosis due to clogged blood vessels by clumpped RBC
Activates compliment partially in periphery.
Agglutinin disintegrates in the warmer parts of the body.
C3b coated RBC are destroyed more in liver and les in spleen.
Doing splencetomy is not useful.
Treatment is keeping the periphery warm.
CAUSE-idiopathic,lymphoma ,mycoplasma pneumoniae,IM
<37 degree centigrade
P component in RBC
sticks to rbc at lower temp in extremities
Activates compliment at warmer tempreture
Discovered in tertiray syphillis by Dr.Donath