This document discusses hemolytic anemia, which is a form of anemia caused by the premature breakdown of red blood cells. The key points are: 1) In hemolytic anemia, the bone marrow compensates for red blood cell loss by increasing red blood cell production up to 10 times normal levels through increased erythropoietin production. 2) Signs of hemolysis include jaundice, dark urine, increased bilirubin in urine, increased LDH, low or absent haptoglobin, and an elevated reticulocyte count on peripheral blood smear. 3) Hemolysis can be intravascular or extravascular. Extravascular causes include splenomegaly while

1. MODERATOR-DR.AMIT VARMA
PRESENTAOR –DR.SUNIL BHATT

2.  Normal shape of RBC- biconcave
 Normal life span of RBC-120 day

3.  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.

4. Compensatory action of bone
marrow
 In response to haemolytic anaemia –dec haemoglobin
 Oxygen supply to kidney is dec,
 Inc erythropoiten from peritubular capillaries around
PCT
 Erythropoiten prevents apoptosis of erthroblast
.causes erythroid hyperplasia
 Bone marrow can inc prod.of RBC 8 to 10 times.
 (compensated haemolytic disease)

5. 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
haemoglobin)
 Slightly less haptoglobin in extravascular
 Very less haptoglobin in intravascular.

6. 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
 Macrocytosis
 Polychromasia(multiple color and shade)
 BONE MARROW
 Hypercelluar bone marrow

8. EXTRAVASCULAR OR
INTRAVASCULAR
extravascular
 SPLENOMEGALY
 intravascular
 dec haptoglobin or absent haptoglobin
 Haemoglobinemia
 Methaemoglobinemia
 Tetrameric to dimer structure of haemoglobin --
endocytosis by PCT cells ---release of iron-- Acute tubular
necrosis –renal failure
 Haemoglobinurea.

11. FEATURES OF EXTRAVASCULAR
HAEMOLYSIS(HERIDITARY
SPHEROCYTOSIS)
PBF
 MCV –DEC
 MCH -NORMAL
 MCHC –HIGH
 RBC SHAPE –SPHERICAL
 RBC COLOR- DARK RED WITH LOSS OF CENTRAL
PALLOR
 OTHER FINDINGS
 SPLENOMEGALY

12. ACUTE OR CHRONIC intravascular
haemolysis
ACUTE
Haemoglobinemia,
methaemoglobinemia,
low haptoglobin
Haemoglobinuria
CHRONIC
Deposition of bilirubin in gall bladder—gall stones.
Splenomegaly.
Haemosiderinurea—due to chronic haemolysis haemosiderin
is formed in PCT.Stained by persian blue.

13. Intrinsic and extrinsic causes
 INTRINSIC CAUSES –cause is related to RBC itself.
 EXTRINSIC CAUSES-where factors external to the
RBC dominate.

14. Intrinsic causes
 INHERITED
 Membrane defects
 Enzymes defect

15. INTRINSIC CAUSES
INHERITED
 Heriditary spherocytosis
 Heriditary elliptosytosis
ACQUIRED
 PNH

16. PNH
 Acquired
 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.
 HAEMOTOLOGICAL CHIMERA
 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
MULTIPLY.
 In severe case pancytopenia

17.  Haemolysis occur all the time
 During sleep occurs more
 Breathing become slow –inc in co2---produce acidic enviornment-alternate
compliment acivated.
 Thrombi formation(hepatic vein,cerebral vein,deep vein of legs)
 Free Hb combine with NO
 Smooth muscle fail to relax—erectile dysfuntion,odynophagia
 Inc infection
 LAP score dec
 TESTS
 HAM TEST
 SUCROSE HAEMOLYSIS TEST
 FLOW CYTOMETRY
 IMMUNOSUPPRESIVE DRUGS
 MONOCLONAL ANTIBODIES—BINDS WITH C5 PREVENTS
BREAKDOWN OF C5 TO A AND B COMPONENT.

18. Enzyme defect
 Hexokinase
 Pyruvate kinase
 G6PD Deficiency
 Glutathione synthese
Glycolysis pathway
disturbed

19.  Haemoglobin synthesis defect
 GLOBIN CHAIN SYNTHESIS IS QUANTITATIVELY REDUCED
 THALASEMIA(ALPHA OR BETA )
 QUALITATIVE DEFECT IN GLOBIN CHAIN
 HAEMOGLOBINOPATHIES
 SICKLE CELL ANAEMIA(GENE defect POSITION NO 6 .GLUTAMIC
ACID IS REPLACED BY VALINE)

20. Extrinsic causes
 Immune mediated causes like
ISO ANTIBODY---ABO ,Rh
AUTO ANTIBODY---
Warm cold agglutinins cold haemolysins

21. WARM ANTIBODY
 IgG
 37 DEGREE CENTIGRADE
 DIRECTED AGAINST -Rh antigen
 Monomeric cannot agglutinate
 Compliment system cannot be activated(RBC has cd59 and
cd55)
 RBC when they pass through spleen converts to
spherocytes.(differentiated by direct coombs test)
 Cause-
idiopathic,lymphoma,leukemia,SLE,drugs(penicillin,cepha
losparin,quinidine,alpha methyl dopa)

22. Cold antibody
 IgM
 Agglutinate
 <37 degree centigrade
 I antigen
 Works only when peripheral parts are exposed to cold
tempreture.
 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

23. Cold haemolysin
 <37 degree centigrade
 IgG
 P component in RBC
sticks to rbc at lower temp in extremities
 Activates compliment at warmer tempreture
 Intravacular haemolysis
 Haemoglobinemia
 Haemoglobinurea
 Discovered in tertiray syphillis by Dr.Donath
LANDSTEINER
 Measles mumps

24. Non immune mediated
 Macro angiopathic haemolytic anaemia
 Mechanical heart valves
 Calcific aortic stenosis
 March haemoglobinuria,bongo drummers
 Micro angiopathic haemolytic anaemia
 Fibrin thrombi --DIC
 Platelet thrombi—TTP,HUS(E COLI O157:H7)
 Splenic sequestration
 Chemical injury
 Infections
 Malaria
 Clostridium-lecithinase

25.  A 40 yr old male comes to us with c/o of
fatigue ,breathlessnesssince 2 months

26. History
 Complains-breathlessness
 fatigue
 fever
 Stool color
 Urine color
 H/O recent blood transfusion,recent
infection,exposure of drugs or toxins,cardiac
prosthesis valve

27. Clinical examination-
Anaemia
Icterus,
Jaundice
Fever
Splenomegaly

28. HOW TO APPROACH

29. CONTINUED….

30. PEREPHERAL BLOOD SMEAR

31.  THANK YOU